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PurposeMethodsResultsConclusionTo review the clinical presentation and diagnostic workup of patients presenting with ocular sarcoidosis as initial manifestation of the disease and identify risk factors for disease recurrence.All cases of sarcoidosis followed in our departments of Ophthalmology and Internal Medicine were retrospectively reviewed. Among 177 patients, 50 had ocular sarcoidosis as initial presentation of the disease. Clinical, biological, radiological, and treatment data were collected. Logistic regression analysis was conducted to identify risk factors for recurrence.Mean age of patients was 51 years, with an equal M/F ratio. Bilateral ocular involvement was present in 86% of cases, with granulomatous panuveitis being the most common manifestation (48%). Two-thirds of patients had extraocular symptoms related to the disease, mostly fatigue. The most frequently involved extraocular site was the supradiaphragmatic lymph nodes, identified in 84% of cases. CRP was normal in 68% of patients, while ACE and lysozyme were elevated in 19% and 64% of cases, respectively. Histopathological confirmation was obtained in 68% of patients. Female sex (odds ratio [OR] 6.66, 95% confidence interval [CI] 1.50–40.40, p = 0.021) and granulomatous panuveitis (OR 7.23, 95% CI 1.70–40.99, p = 0.013) were identified as significant risk factors for disease recurrence.Ocular sarcoidosis is a common initial presentation of systemic sarcoidosis. Diagnosis should be suspected in the presence of bilateral granulomatous panuveitis. Chest CT scan or 18F-FDG PET/CT is mandatory for systemic workup and biopsy is necessary for definite diagnosis. Female sex and a panuveitis presentation are significantly associated with disease recurrence. [ABSTRACT FROM AUTHOR]

Sarcoidosis and tuberculosis (TB) are two granulomatous diseases that often share overlapping clinical features, including uveitis. We measured 368 inflammation-related proteins in serum in both diseases, with and without uveitis from two distinct geographically separated cohorts: sarcoidosis from the Netherlands and TB from Indonesia. A total of 192 and 102 differentially expressed proteins were found in sarcoidosis and active pulmonary TB compared to their geographical healthy controls, respectively. While substantial overlap exists in the immune-related pathways involved in both diseases, activation of B cell activating factor (BAFF) signaling and proliferation-inducing ligand (APRIL) mediated signaling pathways was specifically associated with sarcoidosis. We identified a B-lymphocyte activation signature consisting of BAFF, TNFRSF13B/TACI, TRAF2, IKBKG, MAPK9, NFATC1, and DAPP1 that was associated with sarcoidosis, regardless of the presence of uveitis. In summary, a difference in B-lymphocyte activation is a key discriminative immunological feature between sarcoidosis/ocular sarcoidosis (OS) and TB/ocular TB (OTB). Sarcoidosis patients exhibit higher level of serum B cell activation signature compared to tuberculosis, regardless of uveitis manifestation. [ABSTRACT FROM AUTHOR]

Background: The internet is a common source of health information for patients and caregivers. To date, content and information quality of YouTube videos on sarcoidosis has not been studied. The aim of our study was to investigate the content and quality of information on sarcoidosis provided by YouTube videos. Methods: Of the first 200 results under the search term "sarcoidosis," all English-language videos with content directed at patients were included. Two independent investigators assessed the content of the videos based on 25 predefined key features (content score with 0–25 points), as well as reliability and quality (HONCode score with 0–8 points, DISCERN score with 1–5 points). Misinformation contained in the videos was described qualitatively. Results: The majority of the 85 included videos were from an academic or governmental source (n = 63, 74%), and median time since upload was 33 months (IQR 10–55). Median video duration was 8 min (IQR 3–13) and had a median of 2,044 views (IQR 504 − 13,203). Quality assessment suggested partially sufficient information: mean HONCode score was 4.4 (SD 0.9) with 91% of videos having a medium quality HONCode evaluation. Mean DISCERN score was 2.3 (SD 0.5). Video content was generally poor with a mean of 10.5 points (SD 0.6). Frequently absent key features included information on the course of disease (6%), presence of substantial geographical variation (7%), and importance of screening for extrapulmonary manifestations (11%). HONCode scores were higher in videos from academic or governmental sources (p = 0.003), particularly regarding "transparency of sponsorship" (p < 0.001). DISCERN and content scores did not differ by video category. Conclusions: Most YouTube videos present incomplete information reflected in a poor content score, especially regarding screening for extrapulmonary manifestations. Quality was partially sufficient with higher scores in videos from academic or governmental sources, but often missing references and citing specific evidence. Improving patient access to trustworthy and up to date information is needed. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a multiorgan granulomatous disease that lacks diagnostic biomarkers and targeted treatments. Using blood and skin from patients with sarcoid and non-sarcoid skin granulomas, we discovered that skin granulomas from different diseases exhibit unique immune cell recruitment and molecular signatures. Sarcoid skin granulomas were specifically enriched for type 1 innate lymphoid cells (ILC1s) and B cells and exhibited molecular programs associated with formation of mature tertiary lymphoid structures (TLSs), including increased CXCL12/CXCR4 signaling. Lung sarcoidosis granulomas also displayed similar immune cell recruitment. Thus, granuloma formation was not a generic molecular response. In addition to tissue-specific effects, patients with sarcoidosis exhibited an 8-fold increase in circulating ILC1s, which correlated with treatment status. Multiple immune cell types induced CXCL12/CXCR4 signaling in sarcoidosis, including Th1 T cells, macrophages, and ILCs. Mechanistically, CXCR4 inhibition reduced sarcoidosis-activated immune cell migration, and targeting CXCR4 or total ILCs attenuated granuloma formation in a noninfectious mouse model. Taken together, our results show that ILC1s are a tissue and circulating biomarker that distinguishes sarcoidosis from other skin granulomatous diseases. Repurposing existing CXCR4 inhibitors may offer a new targeted treatment for this devastating disease. [ABSTRACT FROM AUTHOR]

This article discusses a case of a hard lobulated submucosal nodule on the lower lip, which was diagnosed as oral sarcoidosis. The patient presented with a swelling in the lower lip and a family history of cancer. The clinical examination revealed an isolated submucosal nodule with poorly delimited margins and a hardened texture. The diagnosis was confirmed through an incisional biopsy, which revealed multiple granulomas with a sarcoid pattern. The article emphasizes the importance of considering sarcoidosis as a differential diagnosis for oral lesions and highlights the variable clinical presentation of the disease. The treatment for sarcoidosis typically involves systemic corticosteroids, surgical excision, or expectant management. In this case, the oral lesion was completely removed, and the patient was followed up regularly with no signs of recurrence or systemic symptoms. [Extracted from the article]

Background/Objectives: The relationship between sarcoidosis and the occurrence of neoplasia deserves to be investigated, but this relation has been observed in different and heterogeneous populations, leading to conflicting data. To clarify the causal relationship between these two diseases, different risk factors (e.g., smoking), concurrent comorbidities, corticosteroid therapy, and metastasis development—as an expression of cancer aggressiveness—were investigated. Methods: In a retrospective study on 287 sarcoidosis outpatients at the Pneumological Department of the Gemelli Foundation (Rome, Italy) between 2000 and 2024, the diagnosis of cancer was recorded in 36 subjects (12.5%). Results: The reciprocal timeline of the diseases showed three different scenarios: (1) cancer preceding sarcoidosis or sarcoid-like reactions (63.8%); (2) cancer arising after sarcoidosis diagnosis (8.3%); and (3) sarcoidosis accompanying the onset of malignancy (27.8%). Only two subjects with sarcoidosis and cancer showed metastasis, and one of them was affected by lymphoma. Conclusions: These data suggest that granulomatous inflammation due to sarcoidosis may assume an ambivalent role as a "double-edged sword", according to the M1/M2 macrophage polarization model: it represents a protective shield, preventing the formation of metastasis through the induction of immune surveillance against cancer while, on the other hand, it can be a risk factor for carcinogenesis due to the persistence of a chronic active inflammatory status. Low-dose steroid treatment was administered in only 31.6% of the cancer–sarcoidosis subjects for less than six months to control inflammation activity, with no promotive effect on carcinogenesis observed. [ABSTRACT FROM AUTHOR]

Background/Objective: To describe the clinical features and radiological outcomes of patients with spinal cord neurosarcoidosis, treatments, and long-term follow-up for this rare disorder. Methods: A cross-sectional, retrospective medical chart review was performed for all patients with spinal cord neurosarcoidosis treated at a single center between 01/1995 and 12/2020. Radiological imaging, laboratory test results, the type of immunosuppressive therapy, and function test scores were reviewed. Results: We assessed 39 patients with spinal cord neurosarcoidosis (23 men, 16 women, mean age at presentation 46.4 years, SD 10.2 years). The mean (SD) duration of spinal cord neurosarcoidosis at data abstraction was 9.8 (6.3) years. There were 24 patients (62%) with extensive intramedullary lesions, 8 (21%) with multiple patchy intramedullary lesions, 12 (31%) with leptomeningeal involvement, and 7 (18%) with nerve root enhancement. The cervical spine was the most commonly affected region in 33 patients (85%). The most common presenting symptoms were paresthesia/neuropathic pain in 20 (51%) and weakness of extremities in 15 (38%) patients. Most patients (n = 37; 95%) had been treated with corticosteroids at symptom onset, and methotrexate was the most used immunosuppressive therapy (n = 19; 49%). Of 34 patients with follow-up magnetic resonance imaging (MRI) available, the median time to improvement per MRI was 10.8 months (95% CI, 6.1–17.0 months). Of 31 patients with MRI enhancement at presentation, 18 (58%) had complete enhancement resolution at follow-up, with a median time to resolution of 51.8 months (95% CI, 24.9–83.4 months). Patients had significantly lower pyramidal (p = 0.004) and sensory functional (p = 0.031) systems scores from presentation to the last clinic visit. Conclusions: Because spinal cord neurosarcoidosis is challenging to diagnose and no set treatment guidelines exist, clarifying patients' clinical parameters and responses to various treatments is needed to improve timely and efficient care. The incidence of spinal cord involvement in sarcoidosis in our cohort was higher than intracranial involvement and most patients had a long extensive intramedullary lesion. We also observed that most patients with spinal cord neurosarcoidosis improved clinically and radiologically after treatment; however, the resolution of MRI enhancement after immunosuppressive therapy may take years. Prospective studies of neurosarcoidosis will be crucial to address questions about effective treatment and long-term prognosis. [ABSTRACT FROM AUTHOR]

Beryllium is used widely in industry due to its favourable elemental properties. Its extraction and subsequent incorporation into alloys and composites generate hazardous fumes and dust. Chronic beryllium disease (CBD) is an occupational interstitial lung disease that clinically resembles sarcoidosis. We present a case of a 33-year-old jeweller who developed CBD from exposure to dust while grinding unfinished gemstones. While workplace protection exists for beryllium workers, the health risks in artisans are unrecognised. Most cases are diagnosed in industries primarily involved in the use of beryllium (Table I); however, in our case the exposure to beryllium was unexpected. Lymphocyte proliferation testing for beryllium is a key investigation in patients with suspected CBD. To our knowledge, this is the first reported case of CBD diagnosed in a jeweller in South Africa. [ABSTRACT FROM AUTHOR]

A 60-year-old man presented to an outside ophthalmology clinic with 1 month of progressive vision loss in the right eye (OD). Right optic disc edema was noted. Brain and orbit magnetic resonance imaging revealed right optic nerve and left occipital lobe enhancement. He was seen initially by neurology and neurosurgery and subsequently referred to neuro-ophthalmology for consideration of optic nerve biopsy. He was seen 3 months after his initial symptom onset where vision was light perception OD and a relative afferent pupillary defect with optic nerve edema. OS was unremarkable. A lumbar puncture with flow cytometry was negative for multiple sclerosis and lymphoma. At his oculoplastic evaluation for optic nerve biopsy, his vision was noted to be no light perception OD. Optic nerve biopsy demonstrated non-caseating granulomatous inflammation consistent with neurosarcoidosis. The patient was started on high-dose oral steroids with improvement of disc edema, as well as significant improvement in optic nerve and intracranial parenchymal enhancement, although his vision never improved. [ABSTRACT FROM AUTHOR]

• Granulomatous myositis is a severe and difficult to treat disease in which distal motor deficit and clinical amyotrophy seem to be predictive factors for evolution towards severe disability. • A significant proportion of granulomatous myositis cases exhibit clinical or pathological features consistent with inclusion body myositis. • Granulomatous myositis with significant initial hypercalcemia typically show favorable progression with corticosteroid therapy. Granulomatous myositis is a clinical-pathological entity, which has been rarely reported, mostly described in sarcoidosis. Currently, no clear and simple prognostic factor has been identified to predict granulomatous myositis evolution. The clinical, anatomopathological, imaging, and biological characteristics of 26 patients with granulomatous myositis were retrospectively collected to describe clinical presentation and outcomes of this condition. Twenty-six patients with granulomatous myositis were included (14 males) with a median age of symptom onset of 65 years. 54 % of patients presented a severe form of the disease defined as a Rankin score ≥2 at last follow-up visit or a progressive form of the disease (no improvement under treatment). Etiology were sarcoidosis (n = 14), inclusion body myositis (n = 4), autoimmune disease (n = 1), hematological malignancy (n = 1), and idiopathic (n = 6). Distal deficit and amyotrophy were more frequent among those with a severe disease. Corticosteroids led to improvement in 75 % of cases, but 66 % of responders relapsed. Methotrexate appeared as a promising second line therapy with clinical improvement in 50 % of patients, and no relapse in responders. Granulomatous myositis is often a severe and difficult-to-treat disease in which patients frequently progress towards severe disability. The presence of muscle atrophy and distal weakness appears to be frequently associated with a severe form of the disease. [ABSTRACT FROM AUTHOR]

A 74-year-old woman with a history of hypertension and peripheral artery disease and a reported diagnosis of sarcoidosis presents for an episode of syncope and shortness of breath. She had a history of sarcoidosis diagnosed on chest radiography that showed lymphadenopathy. There were no associated symptoms, and she was not previously treated for sarcoidosis. She previously smoked and had quit smoking 9 years earlier. [ABSTRACT FROM AUTHOR]

Sarcoids are the most frequently diagnosed dermatological tumour in horses. It is a disease that can affect various species of equids, such as donkeys, mules and zebras. This type of tumour can develop in all horse breeds, regardless of age and gender. Treatment options depend on many factors, such as the type of lesion, location, extent, owner preference and financial considerations. In the present study, we investigated the TRIM29 expression, the methylation status of its first exon and its involvement in the formation of equine sarcoids. Bisulfite sequencing PCR (BSP) was used to determine DNA methylation at CpG sites and real‐time quantitative polymerase chain reaction (qPCR) was used to detect TRIM29 expression level. Our results showed that TRIM29 is significantly downregulated in lesional samples (FC = −3.72; p < 0.001). Furthermore, TRIM29 expression was significantly correlated (R = −0.73; p < 0.001) with hypermethylation of its specific CpG sites in the first exon of this gene. Our research has demonstrated that the identification of increased methylation of CpG sequences in horse sarcoids, along with the decreased expression of the TRIM29 gene, is an important step towards understanding the molecular mechanisms underlying the disease. These findings can serve in the future as a diagnostic biomarker for horse sarcoids and help in detecting the disease. [ABSTRACT FROM AUTHOR]

This document is a letter published in the Archives of Dermatological Research that discusses a cross-sectional analysis of the All of Us database to understand hypertrophic scars (HTS) in underrepresented groups. The study found that HTS patients were more likely to be Black, Asian, and female, while patients over 65, without health insurance, with lower education, and low income had lower odds of HTS. Patients with HTS also had a higher odds of various comorbidities. The letter includes two tables that provide information on the prevalence of certain characteristics and comorbidities in individuals with HTS compared to those without HTS. The study highlights the importance of early identification of at-risk populations and the need for improved accessibility and affordability of HTS diagnosis and treatment. [Extracted from the article]

Objective: To describe the radiological features of patients with headache as a presenting symptom of neurosarcoidosis. Background: Neurologic complications occur in approximately 5%–10% of patients with sarcoidosis, and approximately 50% of these patients have neurologic deficits at the time sarcoidosis is first diagnosed. A wide spectrum of central and peripheral nervous system clinical manifestations may be observed, including cranial nerve palsies, sensory and/or motor deficits, and headache. Magnetic resonance imaging (MRI) results in patients with neurosarcoidosis may include abnormal contrast enhancement, structural masses, and demyelinating lesions. Methods: This single‐center retrospective cohort study assessed patients who were diagnosed with neurosarcoidosis in an urban tertiary care center between 1995 and 2016. We included patients who had MRI results at the time of diagnosis. Patients were divided into two groups based on the presence or absence of headache as a presenting symptom. The MRI result of meningeal contrast enhancement was reviewed. Results: Of the 110 patients analyzed, 30 (27.3%) had an initial presenting symptom of headache while 80 (72.7%) did not. Patients with headache had a higher proportion of meningeal contrast enhancement on MRI (66.7% [20/30] vs. 25.0% [20/80]; p < 0.001) and leptomeningeal involvement (53.3% [16/30] vs. 7.5% [6/80], p < 0.001) compared to patients with no headache. However, those with headache had a lower proportion of spinal cord localization (13.8% [4/29] vs. 34.2% [26/76], p = 0.038) and intraparenchymal central nervous system involvement (16.7% [5/30] vs. 51.3% [41/80], p = 0.001) compared to patients with no headache. Conclusion: Patients with neurosarcoidosis who presented with headache as an initial symptom had a higher proportion of meningeal contrast enhancement seen by MRI than patients who presented with other neurological symptoms. This suggests a clinico‐radiologic link between headache and meningeal disruption in patients with neurosarcoidosis. [ABSTRACT FROM AUTHOR]

Background: Neurosarcoidosis is a rare entity, usually within the context of systematic sarcoidosis. Isolated neurosarcoidosis and especially a manifestation with pachymeningitis is a notable rarity. Case Report: A 26-year-old patient presented to the emergency department with acute onset, recurrent episodes of occipital headaches spreading over the whole cranium and vomiting without food consumption, for three days. The clinical examination did not reveal any neurological deficits. The laboratory exams showed no pathological findings. A CT examination with angiography did not detect any acute intracranial or vessel pathology. A lumbar puncture was performed to rule out subarachnoid hemorrhage. The results showed a lymphocytic pleocytosis of 400/µL, elevated protein levels of 1077 mg/dL and reduced glucose levels (CSF: 55 mg/dL, Serum: 118 mg/dL). Extensive infectiological examinations did not reveal any signs of infection, including Borrelia spp. and M. tuberculosis. No positive auto-antibodies or vasculitis-related auto-antibodies were detected. The CSF analysis showed negative oligoclonal bands but an isolated increase in β2-microglobulin, neopterin, and IL-2R levels. The MRI examination revealed a dural gadolinium-enhancement, pronounced in the basal cerebral structures and the upper segment of the cervical spine, consistent with neurosarcoidosis. Corticosteroid treatment rapidly led to a significant improvement of the symptoms. No systemic manifestations of sarcoidosis were found. Conclusions: This case report aims to highlight aseptic meningitis with atypical, acute onset headache attacks as a possible manifestation of isolated neurosarcoidosis. Neurosarcoidosis is a clinical entity that requires prompt treatment to avoid permanent neurological deficits. [ABSTRACT FROM AUTHOR]

Background and Objective Methods Results Conclusion Sarcoidosis can manifest with atypical findings on chest computed tomography (CT). Cysts are a rare manifestation of lung sarcoidosis. The aim of the study was to describe a series of patients with cystic sarcoidosis and their clinical‐radiological characteristics and progression.In this retrospective, bicentric study we recruited all patients affected by sarcoidosis with lung cystic lesions at chest CT. We collected clinical characteristics, pulmonary tests and tracked number, distribution and size of the cysts at diagnosis and at the last evaluation.Twelve patients (6 males, median age 53 years) were identified (prevalence: 1.9%; 95% Confidence Interval: 0.8%–2.9%). All patients presented multiple cystic lesions (median number: 14 [range: 2–216]) with a bilateral distribution in 10/12, micronodules and nodules in 11/12 and fibrotic lesions in 4/12. Seven patients had normal lung function test, three had an obstructive syndrome, one had a restrictive syndrome and one had coexistence of both. During follow‐up (median: 10 years [range 1–16 years]), an increase of the number of cysts was observed in four patients. At last evaluation, 3/12 patients experienced a decline of forced vital capacity >10% and 3/12 patients a decline of diffusing capacity for carbon monoxide (DLCO) >10%. A lower DLCO at diagnosis, and the presence of nodules or fibrotic lesions on CT were associated with an increase in the number of cysts.Cystic lung lesions are rare in patients with sarcoidosis and do not influence long term prognosis. [ABSTRACT FROM AUTHOR]

Background: Cough remains a persistent symptom in patients with idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs). To inform future research, treatment and care models, we conducted the first systematic synthesis of evidence on its associated burden. Methods: A literature search was performed for articles published between January 2010 and October 2023 using databases including Embase, MEDLINE and the Cochrane Library. Studies in patients with IPF and other ILDs reporting cough-related measures were eligible for inclusion. Included studies were categorised based on the types of ILD they examined and their design. Study details, patient characteristics and outcomes were extracted, and the risk of bias was assessed. A narrative synthesis approach was employed to interpret the findings. Results: Sixty-one studies were included: 33 in IPF, 18 in mixed-ILDs, six in connective tissue disease-associated-ILDs and four in sarcoidosis. Across the studies, a range of tools to assess cough and its impact were used. The most frequently used measures of cough were cough severity visual analogue scale (VAS) and objective cough counts, whereas the most frequently used health-related quality of life (HRQoL)/impact measures were the St. George's Respiratory Questionnaire (SGRQ) and Leicester Cough Questionnaire (LCQ). In IPF, studies consistently reported correlations between various cough and HRQoL measures, including between cough VAS scores and objective cough counts, LCQ scores and SGRQ scores. Similar correlations were observed in studies in other ILDs, but data were more limited. Qualitative studies in both IPF and other ILDs consistently highlighted the significant cough-related burden experienced by patients, including disruption of daily activities, fatigue and social embarrassment. Although there were no studies specifically investigating the economic burden of cough, one study in patients with fibrotic ILD found cough severity was associated with workplace productivity loss. Conclusions: Our study underscores the heterogeneity in assessing cough and its impact in IPF and other ILDs. The findings confirm the negative impact of cough on HRQoL in IPF and suggest a comparable impact in other ILDs. Our synthesis highlights the need for standardised assessment tools, along with dedicated studies, particularly in non-IPF ILDs and on the economic burden of cough. [ABSTRACT FROM AUTHOR]

Simple Summary: This study analyzed over eighty thousand adverse event reports from the Food and Drug Administration's Adverse Event Reporting System to identify records of patients administered with immune checkpoint inhibitors as monotherapy for various malignancies. We analyzed whether co-occurring viral, bacterial, or fungal infections increased the risk of developing immune-related adverse events, such as pneumonitis, sarcoidosis, myocarditis, nephritis, colitis, hepatitis, and others, in cancer patients treated with those inhibitors and quantified the association. We have found a statistically significant, at the 95% confidence level, association between co-occurring infections and immune-related adverse events in cancer patients treated with immune checkpoint inhibitors. Therapeutic antibodies designed to target three immune checkpoint proteins have been applied in the treatment of various malignancies, including small and non-small cell lung cancers, melanoma, renal cell carcinoma, and others. These treatments combat cancers by reactivating cytotoxic T cells. Nevertheless, this mode of action was found to be associated with a broad range of immune-related adverse events (irAEs), including pneumonitis, sarcoidosis, myocarditis, nephritis, colitis, and hepatitis. Depending on their severity, these irAEs often necessitate the suspension or discontinuation of treatment and, in rare instances, may lead to fatalities. We analyzed over nineteen million reports and identified over eighty thousand adverse event reports from patients treated with immune checkpoint inhibitors submitted to the Food and Drug Administration's Adverse Event Reporting System MedWatch. Reports concerning pembrolizumab, nivolumab, cemiplimab, avelumab, durvalumab, atezolizumab, and ipilimumab revealed a statistically significant association between the irAEs and concurrent infectious diseases for five out of seven treatments. Furthermore, the association trend was preserved across all three types of checkpoint inhibitors and each of the five individual therapeutic agent cohorts, while the remaining two showed the same trend, but an increased confidence interval, due to an insufficient number of records. [ABSTRACT FROM AUTHOR]

Neurosarcoidosis (NS) with hypothalamic-pituitary (HP) involvement (HP-NS) is a rare clinical condition, conferring variable hormonal deficits that are typically irreversible. Here, we present 2 cases of NS with panhypopituitarism. The first patient presented with cauda equina syndrome and arginine vasopressin deficiency, while the second developed recurrent optic neuritis and vision loss in the setting of a sellar mass. In the first case, neurological symptoms resolved after therapy with high-dose glucocorticoids, infliximab, and methotrexate; while in the second, visual restoration followed resection of the granulomatous tissue and immunosuppressive therapy. In both cases, pituitary dysfunction persisted despite neurological improvement. We contextualized the presentations and outcomes through a literature review of HP-NS case reports and case series. This revealed high rates of extraneurologic sarcoidosis in HP-NS patients with panhypopituitarism, while underscoring the need for hormonal replacement--as endocrinopathies rarely respond to sarcoidosis-directed immunosuppression. [ABSTRACT FROM AUTHOR]

Dermoscopy can be an important help for the diagnosis of skin cancers and inflammatory cutaneous diseases. The list of the dermoscopic features reported in granuloma faciale is wide and includes vascular and non‐vascular features. We report here three cases of diffuse flat facial and extrafacial granuloma faciale that exhibited elongated linear vessels simulating branching vessels and diffuse structureless orange areas. The differential diagnosis between flat‐type granuloma faciale, basal cell carcinoma and cutaneous sarcoidosis can be extremely difficult, making histology mandatory before any treatment. [ABSTRACT FROM AUTHOR]

Background: Sarcoidosis is a multisystemic disease that is histologically characterized by non-caseating granulomas in one or more organs. Although hypercalcemia is commonly seen in sarcoidosis, clinically significant hypercalcemia as the initial presentation of sarcoidosis is exceedingly rare. Long-standing hypercalcemia can lead to several complications and needs to be adequately managed to prevent irreversible damage. Currently, there are no standard treatment guidelines for sarcoidosis-induced hypercalcemia, although glucocorticoids have often been used as first-line therapy. Case Report: We describe a 55-year-old male patient who presented with dull right upper quadrant abdominal pain and a 30-pound weight loss over one month. He was found to have severe hypercalcemia, which was treated with intravenous (IV) normal saline and intramuscular calcitonin. Imaging studies revealed hypodense lesions throughout the bilateral hepatic lobes, spleen, and bilateral kidneys, with no pathologic mediastinal, hilar, supraclavicular, or axillary lymphadenopathy or pulmonary parenchymal disease. A splenic biopsy confirmed extrapulmonary sarcoidosis. After initial discharge, the patient was re-admitted weeks later for severe hypercalcemia, which was successfully treated with the initiation of prednisone. Conclusions: In this report, we present an atypical case of isolated extrapulmonary sarcoidosis with severe hypercalcemia as the initial presentation, successfully treated with steroids. [ABSTRACT FROM AUTHOR]

Summary Background Objectives Study design Results Main limitations Conclusions Laser excision is used routinely in the treatment of sarcoids but may be ineffective in cases where complete excision cannot be achieved. A multimodal approach is warranted in these cases. 5‐FU may improve the lethal effect of cryosurgery as an adjunct to laser excision.To compare two treatment protocols for equine sarcoids, laser excision alone versus a combination protocol of laser excision, cryosurgery and 5‐FU chemotherapy. Factors associated with sarcoid recurrence are also investigated.Retrospective case controlled study.Eighty‐four horses with 168 histologically confirmed sarcoids were included, with a median follow‐up time of 39 months (IQR 21–62 months). Sarcoid recurrence at the treated site was reported in 38% of cases and in 23% of any individual sarcoid. No significant difference was demonstrated between treatment categories in either rate of sarcoid recurrence (p = 0.45 for any treated horse, p = 0.63 for individual sarcoid) or time to sarcoid recurrence (p = 0.73). Sarcoid recurrence was higher in horses with a greater number of sarcoids (OR 1.2 (1.0–1.5), p = 0.03); when treatment had been received prior to admission (OR 7.6 (2.0–33), p = 0.004). Horses with urogenital sarcoids or >1 mixed sarcoid experienced more rapid recurrence (HR 3.6 (1.3–10), p = 0.02 and HR 9.9 (3.3–30), p < 0.001) and recurrence was less rapid following the treatment of a horse's first sarcoid (HR 0.3 (0.1–0.7), p = 0.009).Significant differences in case populations in each treatment category. Treatment selection was neither blinded nor randomised and missing data and recall bias limit the study's power. Sarcoid recurrence was owner reported.When assessing the likelihood of sarcoid recurrence, characteristics of both the individual patient and sarcoid phenotype must be considered carefully when selecting a specific treatment protocol. [ABSTRACT FROM AUTHOR]

Summary Equine sarcoidosis (ES) is a rare disease characterised by granulomatous inflammation of one or multiple organs. The skin is most frequently affected in horses and the clinical presentation can range from scaling and crusting lesions to nodular or tumour‐like masses. ES has been divided into three subtypes depending on the distribution of lesions in the body: the localised, partially generalised and generalised form. Overall, no breed or sex predilections have been consistently observed so far. Most affected horses are older than 3 years although the disease may occur at any age. While aetiology and pathogenesis of ES have not yet been fully determined, a chronic exaggerated immune response to one specific or multiple antigens is frequently hypothesised. Diagnosis is based upon history, clinical presentation, histopathology and exclusion of likely differential diagnoses. The treatment of choice in ES is initial anti‐inflammatory to immunosuppressive doses of corticosteroids with subsequent tapering doses. Reported treatment success seems to be highly variable and prospective studies on the efficacy of corticosteroids or other agents are currently lacking in ES. Prognosis for survival is good for the localised form and poor for the other forms of ES. Complete remission of lesions in the localised form following treatment is less frequent and response to treatment is typically characterised by a slowdown or cessation of disease progression. There are scarce reports of spontaneous remission of this disease. [ABSTRACT FROM AUTHOR]

Community advisory boards (CABs) are increasingly recognized as a means of incorporating patient experience into clinical practice and research. The power of CABs is derived from engaging with community members as equals throughout the research process. Despite this, little is known of community member experience and views on best practices for running a CAB in a rare pulmonary disease. What are CAB members' views on the best practices for CAB formation and maintenance in a rare pulmonary disease? In August 2021, we formed the Cleveland Clinic Sarcoidosis Health Partners (CC-HP) as a CAB to direct research and clinic improvement initiatives at a quaternary sarcoidosis center. We collaboratively evaluated our process for formation and maintenance of the CC-HP with the patient members of the group. Through the series of reflection/debriefing discussions, CAB patient members developed a consensus account of salient obstacles and facilitators of forming and maintaining a CAB in a rare pulmonary disease. Clinician and community members of the CC-HP found published guidelines to be an effective tool for structuring formation of a CAB in a rare pulmonary disease. Facilitators included a dedicated coordinator, collaborative development of projects, and a focus on improving clinical care. Obstacles to CAB functioning were formal structure, focus on projects with academic merit but no immediate impact to patients, and overreliance on digital resources. By centering our evaluation of our CAB on community member experience, we were able to both identify facilitators and impediments to CAB as well as improve our own processes. [ABSTRACT FROM AUTHOR]

Chronic beryllium disease (CBD), or berylliosis, is an interstitial lung disease caused by the chronic inhalation of finely particulate beryllium, frequently mistaken for sarcoidosis. It is rarely associated with skin nodular lesions, asymptomatic granulomatous hepatitis or calcium nephrolithiasis. To date, it has never been reported as a diffused multi-organ granulomatous disease. A 60-year-old Pakistani man, a former excavation worker with ancient history of suspected sarcoidosis, underwent a left nephroureterectomy for suspected papillary kidney carcinoma. The histopathological analysis showed a benign non-necrotic granulomatous infiltration of the renal pelvis and ureter. Six months later, he suffered from two consecutive episodes of acute kidney failure. Bladder biopsies found similar noncaseous granulomatosis and kidney biopsies showed interstitial nephritis. Known for suspected asthma, sleep apnea, and usual interstitial pneumonia, the patient would regularly consult for episodes of pyrexia, chills, nocturnal coughing, and wheezing. As kidney function gradually worsened, he ultimately started hemodialysis and was transferred to our facility. A positive blood beryllium lymphocyte proliferation test confirmed the diagnosis of CBD. This original report is the first description of multi-organ berylliosis with diffused urothelial granulomatosis and pseudo-tumor. The patient's pulmonary disease is minimal compared with renal and urinary tract involvement, eventually responsible for end-stage kidney disease. Berylliosis usually responds to glucocorticoids. This case report highlights the importance of evoking the diagnosis of CBD in the presence of any granulomatosis, even extra-thoracic, especially if associated with pulmonary symptoms, however atypical. [ABSTRACT FROM AUTHOR]

Objective: Sarcoidosis is a chronic inflammatory disease that affects many organs and systems, especially the lungs, lymph nodes and musculoskeletal system. Musculoskeletal system involvement patterns in sarcoidosis are not well defined and literature data are limited in terms of clinical features. This study aimed to investigate the clinical and demographic characteristics of sarcoidosis patients with musculoskeletal system involvement. Methods: One hundred and four patients with sarcoidosis who were followed up in our clinic between June 2020 and January 2024 were evaluated retrospectively. Forty-four sarcoidosis patients aged >18 years with objectively detected musculoskeletal system involvement were included in the study. The musculoskeletal system involvement patterns, clinical and laboratory findings, and demographic characteristics of the patients were analyzed. Results: Musculoskeletal involvement was found in 44 of the 104 sarcoidosis patients (42%). Of the 44 patients with musculoskeletal involvement, 37 (77.3%) were female, the mean age was 46.9±12.2 years, and the mean follow-up time was 14±11.8 months. The most common objective musculoskeletal pathology was chronic arthritis (17%), mostly in the form of chronic oligoarthritis. Wrist (50% vs. 3.8%, p=0.001) and metacarpophalangeal joint involvement (38.9% vs. 0, p=0.001) were significantly higher in patients with chronic arthritis than in patients with acute arthritis. While joint involvement starting as monoarthritis was an independent negative predictor for the development of chronic arthritis [odds ratio (OR): 0.046, 95% confidence interval (CI): 0.003-0.815, p=0.036], the presence of obesity at baseline was a positive predictor for the development of chronic arthritis (OR: 11.968, 95% CI: 1.069-133.960, p=0.044). Conclusion: Sarcoidosis can present with a wide range of musculoskeletal manifestations. The most common musculoskeletal pathologies in sarcoidosis are acute and chronic arthritis. Sarcoidosis patients presenting with monoarthritis do not usually develop chronic arthritis. Patients with chronic sarcoid arthritis initially present more hand-joint involvement. Obesity is a risk factor for the development of chronic arthritis. [ABSTRACT FROM AUTHOR]

Although the role of T lymphocytes in sarcoidosis (SA) and lung cancer (LC) is quite well reported, the occurrence of B cells in disease microenvironments may suggest their potential role as natural modifiers of the immune response. The aim of this study was to investigate the B-cell profile and lymphocyte-related hematological parameters between patients with SA, LC and healthy controls (HCs). The cells were assessed by flow cytometry and a hematological analyzer in peripheral blood (PB) and material from lymph nodes (LNs) obtained by the EBUS/TBNA method. We showed that in SA patients, there were higher percentages of naïve B and CD21low B cells and a lower percentage of class-switched memory B cells than LC patients in LNs. We observed a higher median proportion of non-switched memory and transitional B cells in the PB of SA patients than in LC patients. We noticed the lowest median proportion of class-switched memory B cells in the PB from SA patients. LC patients had a higher percentage of RE-LYMP and AS-LYMP than SA patients. Our study presented a different profile of B-cell subpopulations in SA and LC patients, distinguishing dominant subpopulations, and showed the relocation from distant compartments of the circulation to the disease microenvironment, thus emphasizing their role. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Sarcoidosis is a systemic granulomatous disease with an unknown cause, marked by the presence of noncaseating granulomas in the affected organs. While the pulmonary interstitium is most frequently involved, the disease can affect almost any other organ system. Extrapulmonary involvement can occur with or without lung involvement, but isolated extrapulmonary involvement is a rare event. Isolated splenomegaly is very rare and presents an uncommon manifestation of sarcoidosis, its diagnoses is a challenge due to a broad differential diagnosis. Here, we present an intriguing case of a 28‐year‐old male with isolated splenic sarcoidosis. Sarcoidosis is a systemic disease of unknown cause, marked by the presence of noncaseating granulomas in affected organs. It most frequently impacts the pulmonary interstitium, though it can also affect nearly any other organ system. This involvement can occur with or without lung involvement, but isolated extrapulmonary cases are observed in only about 10% of instances. Furthermore, isolated splenomegaly is an exceptionally rare event and an uncommon presentation of sarcoidosis, posing a significant clinical challenge due to the wide differential diagnosis. Potential differential diagnoses include hematologic cancers, primary or metastatic splenic tumors, infiltrative diseases, inflammatory conditions, and infections. We present a noteworthy case of a 28‐year‐old with isolated splenic sarcoidosis. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Long COVID, often following SARS‐CoV‐2 infection, may stem from sustained inflammation, overlapping with autoimmune diseases like sarcoidosis. Though specific treatments lack, this link could shape future diagnostic and therapeutic methods. [ABSTRACT FROM AUTHOR]

Introduction Methods Results Conclusion In patients with cardiac sarcoidosis (CS), implantable cardioverter‐defibrillators (ICDs) are important for preventing sudden cardiac death. This study aimed to investigate sex disparities in CS patients undergoing ICD implantation.The 2016–2020 National Inpatient Sample (NIS) database compared the characteristics and outcomes of males and females with CS receiving ICDs.Among 760 CS patients who underwent inpatient ICD implantation, 66.4% were male. Males were younger (55.0 vs. 56.9 years, p < .01), had higher rates of diabetes (31.7% vs. 21.6%, p < .01) and chronic kidney disease (CKD) (16.8% vs. 7.8%, p < .01) but lower prevalence of atrial fibrillation (AF) (11.9% vs. 23.5%, p < .01), sick sinus syndrome (4.0% vs. 7.8%, p = .024), ventricular fibrillation (VF) (9.9% vs. 15.7%, p = .02), and black ancestry (31.9% vs. 58.0%, p < .01). Unadjusted major adverse cardiovascular events (MACE), defined as a composite of in‐hospital death, myocardial infarction (MI), and ischemic stroke, was higher in females (11.8% vs. 6.9%, p = .024), but when adjusted for age and tCharlson Comorbidity Index (CCI), females demonstrated significantly lower odds of experiencing MACE (aOR: 0.048, 95% CI: 0.006–0.395, p = .005). Incidence of acute kidney injury (AKI) post‐ICD was significantly lower in females (15.7% vs. 23.8%, p = .01) as was the adjusted odds (aOR: 0.282, 95% CI: 0.146–0.546, p < .01). There was comparable mean length of stay and hospital charges.ICD utilization in CS patients is more common among males, who have a higher prevalence of diabetes and CKD but a lower prevalence of AF, sick sinus syndrome, and VF. Adjusted MACE and AKI were significantly lower in females. [ABSTRACT FROM AUTHOR]

Background: Sarcoidosis is a heterogeneous granulomatous disease with no accurate biomarkers of disease progression. Therefore, we profiled and integrated the DNA methylome, mRNAs, and microRNAs to identify molecular changes associated with sarcoidosis and disease progression that might illuminate underlying mechanisms of disease and potential biomarkers. Methods: Bronchoalveolar lavage cells from 64 sarcoidosis subjects and 16 healthy controls were used. DNA methylation was profiled on Illumina HumanMethylationEPIC arrays, mRNA by RNA-sequencing, and miRNAs by small RNA-sequencing. Linear models were fit to test for effect of sarcoidosis diagnosis and progression phenotype, adjusting for age, sex, smoking, and principal components of the data. We built a supervised multi-omics model using a subset of features from each dataset. Results: We identified 1,459 CpGs, 64 mRNAs, and five miRNAs associated with sarcoidosis versus controls and four mRNAs associated with disease progression. Our integrated model emphasized the prominence of the PI3K/AKT1 pathway, which is important in T cell and mTOR function. Novel immune related genes and miRNAs including LYST, RGS14, SLFN12L, and hsa-miR-199b-5p, distinguished sarcoidosis from controls. Our integrated model also demonstrated differential expression/methylation of IL20RB, ABCC11, SFSWAP, AGBL4, miR-146a-3p, and miR-378b between non-progressive and progressive sarcoidosis. Conclusions: Leveraging the DNA methylome, transcriptome, and miRNA-sequencing in sarcoidosis BAL cells, we detected widespread molecular changes associated with disease, many which are involved in immune response. These molecules may serve as diagnostic/prognostic biomarkers and/or drug targets, although future testing is required for confirmation. [ABSTRACT FROM AUTHOR]

Purpose Methods Results Conclusion To evaluate possible risk factors for uveitis among Finnish sarcoidosis patients.Patient charts of patients with sarcoidosis, with (n = 97) or without (n = 255) uveitis, and with a comprehensive eye examination from January 2014 to January 2021 at Tays Eye Centre, Tampere University Hospital, Finland were studied.Sarcoidosis patients with uveitis had higher rate of lymphocytopenia (43% vs. 29%, p = 0.041) and lower serum lysozyme levels (2.0 mg/L vs. 2.3 mg/L, p = 0.049; 95% CI, −0.692 to −0.002). Lysozyme level or lymphocytopenia did not have a statistically significant effect on the probability of uveitis in a binary logistic regression analysis. No other differences in the potential risk factors with p‐values ≤0.05 were found, including bilateral hilar lymphadenopathy, serum angiotensin‐converting enzyme (ACE) levels, sex, age and history of smoking.Lymphocytopenia and lower serum lysozyme levels present as possible risk factors for uveitis among patients with sarcoidosis. Systematic measurement of lymphocyte and lysozyme levels in sarcoidosis is needed to further understand their role as potential risk factors. [ABSTRACT FROM AUTHOR]

This article discusses a case of chronic nonbacterial osteomyelitis (CNO) complicated by recurrent erythema nodosum (EN) and its successful treatment with salazosulfapyridine (SASP). CNO is a condition characterized by bone lesions and can also present with mucocutaneous symptoms. EN is a skin condition associated with inflammation of the subcutaneous tissue. The patient initially received naproxen for treatment but experienced persistent symptoms. SASP was then introduced and led to remission of both CNO and EN. The use of SASP as a second-line treatment for refractory CNO and EN is suggested. [Extracted from the article]

Objectives To characterize joint involvement (JI) in sarcoidosis, a systematic search of MEDLINE, EMBASE and Cochrane Library was conducted from inception to July 2022 for publications reporting its prevalence, pattern, treatment and outcome. Methods The pooled prevalence estimates (PPE) with 95% CI were calculated using binomial distribution and random effects. Meta-regression method was used to examine factors affecting heterogeneity between studies. Results Forty-nine articles were identified comprising a total of 8574 sarcoidosis patients, where 12% presented with JI (95% CI 10, 14; I 2 = 0%). The PPE for sarcoid arthritis (SA) was 19% (95% CI 14, 24; I 2 = 95%), and 32% (95% CI 13, 51; I 2 = 99%) for arthralgia. Heterogeneity was due to higher JI prevalence reported in Western Asia and the Middle East, in rheumatology clinics and via surveys. Sample size of SA varied from 12 to 117 cases. Ankles were most frequently affected (PPE 80%) followed by knees and wrists. Monoarthritis was uncommon (PPE 1%; 95% CI 0, 2; I 2 = 55%). Acute SA prevailed (PPE 79%; 95% CI 72, 88; I 2 = 69%) with an equal proportion of oligo and polyarthritis and was frequently accompanied by erythema nodosum (PPE 62%; 95% CI 52, 71; I 2 = 16%). Chronic SA was predominantly polyarticular with a higher frequency of the upper extremity joints affected. Most common non-articular manifestations with SA included fever (52%), erythema nodosum (41%), hilar adenopathy (86%) and interstitial lung disease (23%) of which one-third required corticosteroids and/or immunosuppressants. Conclusion SA occurred early in the disease with a PPE of 19% and most frequent pattern of acute oligo- or polyarthritis predominantly affecting the lower extremity large joints. [ABSTRACT FROM AUTHOR]

This review article delves into the complexities of granuloma formation, focusing on the metabolic reprogramming within these immune structures, especially in tuberculosis and sarcoidosis. It underscores the role of the monocyte–macrophage lineage in granuloma formation and maintenance, emphasizing the adaptability of these cells to environmental cues and inflammatory stimuli. Key to the discussion is the macrophage polarization influenced by various cytokines, with a detailed exploration of the metabolic shifts towards glycolysis under hypoxic conditions and the utilization of the pentose phosphate pathway (PPP) for crucial biosynthetic processes. Significant attention is given to the metabolism of L-arginine in macrophages and its impact on immune response and granuloma function. The review also highlights the role of mechanistic target of rapamycin (mTOR) signaling in macrophage differentiation and its implications in granulomatous diseases. Discoveries such as elevated PPP activity in granuloma-associated macrophages and the protective role of NADPH against oxidative stress offer novel insights into granuloma biology. The review concludes by suggesting potential therapeutic targets within these metabolic pathways to modulate granuloma formation and function, proposing new treatment avenues for conditions characterized by chronic inflammation and granuloma formation. This work contributes significantly to the understanding of immune regulation and chronic inflammation, presenting avenues for future research and therapy in granulomatous diseases. [ABSTRACT FROM AUTHOR]

Chronic inflammation is increasingly recognized as a risk factor for VTE, but unlike other inflammatory diseases including systemic lupus erythematosus and rheumatoid arthritis, data on the risk of VTE in patients with sarcoidosis are sparse. Do patients with sarcoidosis have a higher long-term risk of VTE (pulmonary embolism or DVT, and each of these individually) compared with the background population? Using Danish nationwide registries, patients aged ≥ 18 years with newly diagnosed sarcoidosis (two or more inpatient/outpatient visits, 1996-2020) without prior VTE were matched 1:4 by age, sex, and comorbidities with individuals from the background population. The primary outcome was VTE. We included 14,742 patients with sarcoidosis and 58,968 matched individuals (median age, 44.7 years; 57.2% male). The median follow-up was 8.8 years. Absolute 10-year risks of outcomes for patients with sarcoidosis vs the background population were the following: VTE, 2.9% vs 1.6% (P <.0001), pulmonary embolism, 1.5% vs 0.7% (P <.0001), and DVT, 1.6% vs 1.0% (P <.0001), respectively. In multivariable Cox regression, sarcoidosis was associated with an increased rate of all outcomes in the first year after diagnosis (VTE: hazard ratio [HR], 4.94; 95% CI, 3.61-6.75) and after the first year (VTE: HR, 1.65; 95% CI, 1.45-1.87) compared with the background population. These associations persisted when excluding patients with a history of cancer and censoring patients with incident cancer during follow-up. Three-month mortality was not significantly different between patients with VTE with and without sarcoidosis (adjusted HR, 0.84; 95% CI, 0.61-1.15). In this nationwide cohort study, sarcoidosis was associated with a higher long-term risk of VTE compared with a matched background population. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Modalities to improve tissue acquisition during endobronchial ultrasound‑guided transbronchial needle aspiration (EBUS‑TBNA) have been investigated. Endobronchial ultrasound‑guided transbronchial mediastinal cryobiopsy (EBUS‑TMC) is a modality to obtain larger histological samples by inserting a cryoprobe into the mediastinal lesion. We aimed to study the diagnostic yield and safety of EBUS‑TMC. Methods: We performed a systematic search of the PubMed and Embase databases to extract the relevant studies. We then performed a meta‑analysis to calculate the diagnostic yield of EBUS‑TMC and compare it with EBUS‑TBNA. Results: Following a systematic search, we identified 14 relevant studies (869 patients undergoing EBUS‑TMC and EBUS‑TBNA). We then performed a meta‑analysis of the diagnostic yield of EBUS‑TMC and EBUS‑TBNA from studies wherein both procedures were performed. The pooled diagnostic yield of EBUS‑TMC was 92% (95% confidence interval [CI], 89%–95%). The pooled diagnostic yield of EBUS‑TBNA was 81% (95% CI, 77%–85%). The risk difference in yield was 11% (95% CI, 6%–15%, I 2 = 0%) when EBUS‑TMC and EBUS‑TBNA were compared. The only complication reported commonly with EBUS‑TMC was minor bleeding. The complication rate was comparable with EBUS‑TBNA. Conclusion: EBUS‑TMC provides a greater diagnostic yield with a similar risk of adverse events compared to EBUS‑TBNA. Future studies are required to clearly establish which patients are most likely to benefit from this modality. [ABSTRACT FROM AUTHOR]

Non-resolving consolidation refers to the persistence of radiographic abnormalities beyond the anticipated timeframe. Conditions such as infection, malignancy, inflammatory disorders, and connective tissue diseases can all manifest as non-resolving consolidation. We share a clinicopathological case study involving a 30-year-old male who exhibits nonresolving consolidation accompanied by hepatosplenomegaly. [ABSTRACT FROM AUTHOR]

Background: Neurosarcoidosis is rare, and among its manifestations, nerve root involvement has been reported in only a few cases. Therefore, magnetic resonance imaging (MRI) findings of neurosarcoidosis, particularly those involving nerve roots, are scarce in the literature. Methods: We presented the case of neurosarcoidosis involving cervical nerve roots and cranial nerves, alongside a systematic literature review. Results: A 28-year-old female suddenly developed right facial numbness as well as left upper extremity and left hand pain. Initial brain and spine MRI showed a bulging mass of T2 iso-to-high signal intensity in the left Meckel's cave/trigeminal nerve, as well as diffuse enlargement of the right C6 and C7 nerve roots. Follow-up MRI at 2 months revealed a reduction in the size of the initial lesion and the appearance of new similar lesions on the contralateral side (right Meckel's cave, left C3–C8 nerve roots). In particular, the lesions involving the nerve roots demonstrated central enlargement along the nerve roots, without involvement of the adjacent spinal cord. All these lesions exhibited enhancement, leading to the differentiation between sarcoidosis and lymphoma. Sarcoidosis was subsequently confirmed through biopsy of a hilar lymph node. Conclusions: This report presents a distinctive MRI feature of neurosarcoidosis involving spinal nerve roots, representing the first of its kind, and describes the evolution of MRI findings throughout the clinical course. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Pericardial sarcoidosis is an uncommon cause of chest pain to consider, and it requires a heightened level of suspicion and thorough history gathering. If there is suspicion of inflammatory disease, pursuing advanced imaging and biopsies is crucial, as early immunosuppressive treatment can enhance outcomes. Pericardial involvement in sarcoidosis is a rare condition with limited research. This case study discusses a 52‐year‐old African American woman who presented with subacute chest pain and was diagnosed with pericardial sarcoidosis. Diagnostic evaluation revealed extensive lymphadenopathy and pericardial effusion, and a pericardial biopsy confirmed non‐caseating granulomatous inflammation. Treatment with steroids and methotrexate resulted in clinical improvement. Eight months follow‐up showed near resolution of pericardial disease. This case emphasizes the importance of considering cardiac sarcoidosis in sarcoidosis patients, utilizing advanced imaging for accurate diagnosis, and tailoring treatment to the level of cardiac involvement. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Sarcoidosis‐induced LETM represents a rare but life‐threatening neurological manifestation of sarcoidosis, characterized by spinal cord inflammation, and associated neurological deficits. Sarcoidosis should be included in the differential diagnosis of LETM, particularly in patients with no lung involvement. Prompt recognition and management are obligatory to optimize outcomes and prevent long‐term disability. Sarcoidosis is a multisystem inflammatory granulomatous disorder characterized by the formation of noncaseating granulomas. Although sarcoidosis commonly affects the skin, lymph nodes, and lungs, neurological involvement of sarcoidosis has also been reported. Longitudinally extensive transverse myelitis (LETM) is a rare but well‐documented serious manifestation of neuroscoidosis. We report a case of LETM caused by sarcoidosis in a 53‐year‐old male who presented with progressive bilateral lower extremity weakness, urinary retention, and paresthesia. Laboratory evaluations revealed elevated inflammatory markers. Magnetic resonance imaging of the spine showed hyperintense signals consistent with transverse myelitis. Cerebrospinal fluid analysis revealed lymphocytic pleocytosis and elevated protein levels. Chest computed tomography showed hilar lymphadenopathy. A biopsy of the intrathoracic lymph node showed noncaseating granulomas consistent with sarcoidosis. A diagnosis of sarcoidosis‐induced LETM was made after ruling out all other possible etiologies. His condition improved gradually after starting high‐dose prednisone, mycophenolate, and rehabilitation strategies. Our case underscores the importance of prompt diagnosis and management of sarcoidosis‐induced LETM and highlights that sarcoidosis must be included among differential diagnoses of LETM, especially in cases with no lung involvement. [ABSTRACT FROM AUTHOR]

Key clinical message: Isolated cardiac sarcoidosis may rarely present with TIA or stroke as an initial clinical manifestation. This case highlights the necessity of a broad differential and a high degree of suspicion for cardiac sarcoidosis in a patient with new neurologic symptoms and evidence of cardiac disease. Cardiac sarcoidosis is a rare disease with a variety of clinical manifestations including heart failure and sudden death. Stroke as the earliest sign of disease has been described in rare cases. We present a case of a 54‐year‐old female with recurrent transient ischemic attacks (TIAs) of unknown etiology, initially in the absence of left ventricular dysfunction. Cardiomyopathy was later identified on echocardiography after a second TIA. Cardiac MRI was remarkable for focal left ventricular wall thinning with akinesis and dyskinesis of multiple wall segments, a right ventricular aneurysm, and diffuse myocardial late gadolinium enhancement. PET/CT showed multifocal areas of myocardial FDG uptake. At follow‐up, echocardiography showed a left ventricular apical thrombus, in a previously identified thinned, akinetic region, suggesting cardioembolic origin for previous TIAs. She was started on anticoagulation therapy, prednisone, methotrexate, and adalimumab, with resolution of the thrombus and improvement in cardiac function. In conclusion, this case highlights the need to consider CS as a potential cause of cerebrovascular ischemic events in patients with few stroke risk factors but findings indicative of cardiac disease. It is essential to further explore the mechanisms behind these events and develop treatments that target their causes in this patient population. [ABSTRACT FROM AUTHOR]

Tumour necrosis factor-α (TNF- α) antagonists are considered a significant therapeutic option in the treatment of sarcoidosis. Nevertheless, their use can also paradoxically result in sarcoidosis-like reactions. Here, we present a case of a 56-year-old patient with psoriatic arthritis who after 3 months of certolizumab therapy developed pulmonary sarcoidosis. Therefore, certolizumab was discontinued and prednisone initiated. Subsequently, 4 months later a complete remission of interstitial lesions was observed. Due to insufficient control of psoriatic arthritis, upadacitinib and methotrexate were prescribed and despite initial improvement, a couple of months later a massive exacerbation of skin psoriasis occurred and the treatment was switched to secukinumab. As of today, no evidence of sarcoidosis recurrence has been noted. Drug-induced sarcoidosis-like reactions (DISR) appear to be less frequently associated with certolizumab rather than with other anti-TNF-α agents. However, specific mechanisms of this phenomenon remain unclear and require future investigation. [ABSTRACT FROM AUTHOR]

A 76-year-old male diagnosed with sarcoidosis presented with atraumatic left anterior knee pain. Initial imaging of the left lower extremity revealed an eccentrically-based lytic lesion in the mid-distal femur with cortical erosion and an additional lytic lesion in the proximal tibia. Magnetic resonance imaging (MRI) demonstrated an aggressive lesion in the proximal tibia with surrounding marrow edema, cortical breach, and erosion into the distal patellar tendon. Given concern for metastatic bone lesions, a18-fluorodeoxyglucose positron emission tomography/computed tomography scan (FDG PET/CT) was performed which demonstrated concordant hypermetabolic lytic lesions at the left mid-distal femur and the left proximal tibia, as well as hypermetabolic diffuse lymphadenopathy. The patient was presumed to have metastatic lung cancer based on the presence of lung nodules. Due to concern for impending pathologic fracture, the patient underwent open biopsy with a plan for prophylactic fixation of both lesions. Intra-operatively, however, both lesions were found to contain pus, from which cultures ultimately grew Cryptococcus neoformans. This is a case of disseminated skeletal cryptococcosis masquerading as metastatic cancer in a patient without classic risk factors for disseminated cryptococcosis (defined as extrapulmonary evidence of infection). Classically, disseminated cryptococcosis is thought to occur in severely immunocompromised patients, such as those with human immunodeficiency virus (HIV) or organ transplant recipients. This case highlights the need to maintain a high index of suspicion in patients with underlying immunocompromising conditions, including less common conditions such as sarcoid, who present with bony lesions. This case report then discusses the diagnostic evaluation and treatment of disseminated skeletal cryptococcosis. [ABSTRACT FROM AUTHOR]

Sarcoidosis is a chronic granulomatous disease predominantly affecting the lungs and inducing significant morbidity and elevated mortality rate. The etiology of the disease is unknown but may involve exposure to an antigenic agent and subsequent inflammatory response resulting in granuloma formation. Various environmental and occupational risk factors have been suggested by previous observations, such as moldy environments, insecticides, and bird breeding. Our study investigated the association of air pollution with diagnosis of sarcoidosis using a case-control design. Penn State Health electronic medical records from 2005 to 2018 were examined for adult patients with (cases) and without (controls) an International Classification of Disease (ICD)-9 or −10 code for sarcoidosis. Patient addresses were geocoded and 24-hr residential-level air pollution concentrations were estimated using spatio-temporal models of particulate matter <2.5 μm (PM2.5), ozone, and PM2.5 elemental carbon (EC) and moving averages calculated. In total, 877 cases and 34,510 controls were identified. Logistic regression analysis did not identify significant associations between sarcoidosis incidence and air pollution exposure estimates. However, the odds ratio (OR) for EC for exposures occurring 7–10 years prior did approach statistical significance, and ORs exhibited an increasing trend for longer averaging periods. Data suggested a latency period of more than 6 years for PM2.5 and EC for reasons that are unclear. Overall, results for PM2.5 and EC suggest that long-term exposure to traffic-related air pollution may contribute to the development of sarcoidosis and emphasize the need for additional research and, if the present findings are substantiated, for public health interventions addressing air quality as well as increasing disease surveillance in areas with a large burden of PM2.5 and EC. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionUveitis is a common ocular manifestation in individuals with sarcoidosis, a multisystem inflammatory disorder. This study aimed to explore clinical and genetic factors associated with the presence or absence of uveitis in sarcoidosis patients.Total 625 Dutch sarcoidosis patients were included. Among these, 170 underwent ophthalmic examination, and 61 were diagnosed with uveitis. Demographic and clinical data, including age, gender, race, biopsy status, chest radiography findings, TNF-α inhibitor treatment, and uveitis classification were collected retrospectively from medical records. Genetic data was available for HLA haplotypes, TNF-α G-308A, and BTNL2 G16071A polymorphisms.The majority of the patients presented with bilateral uveitis (80.3%). The proportion of women was higher in the uveitis group compared to the non-uveitis group (67.2% and 47.7%; p = 0.014). Pulmonary involvement (chest radiographic stage II-III) was significantly lower in patients with uveitis (36.1% versus 64.2%; p < 0.001). Patients with uveitis were more often treated with TNF-α inhibitors (67.2% versus 29.4%; p < 0.001) and the outcome was better compared with the non-uveitis group, 92% vs 68%, responders (p < 0.012). Uveitis patients treated with TNF-α inhibitors (either adalimumab or infliximab) were more likely to suffer from intermediate or posterior uveitis than anterior uveitis. Genetic analysis identified a significant association between the BTNL2 G16071A GG genotype and uveitis (p = 0.012).This study highlights distinctive demographic, clinical and genetic features associated with uveitis in sarcoidosis patients. Ocular sarcoidosis was more prevalent in women. Further research is warranted to explore the implications of these findings for treatment strategies and prognostic assessments. [ABSTRACT FROM AUTHOR]

Background/Objectives: Pulmonary hypertension (PH) often accompanies chronic lung diseases. Several chronic lung diseases with PH portends unfavorable outcomes. We investigated which variables in this cohort of patients with chronic lung disease and PH predicts mortality. Methods: This is a retrospective analysis of patients with chronic lung disease and PH at a single tertiary, academic center. The underlying lung disease included were COPD, IPF, other fibrotic ILD, non-fibrotic ILD, fibrotic sarcoidosis, and CPFE. All patients had right heart catheterization diagnostic of PH as well as pulmonary function testing data including 6 min walk testing. Univariable and multivariate Cox regression was performed to identify variables associated with mortality. Results: We identified 793 patients with chronic lung disease and PH. In total, 144 patients died prior to potential lung transplant. In multivariable Cox regression IPF, other fibrotic ILD, non-fibrotic ILD, and CPFE were significantly associated with an increased risk of mortality. Severe PH (PVR > 5 WU), FEV1 < 30% predicted, FVC < 40% predicted, 6 min walk distance < 150 m were also significantly associated with an increased risk of mortality. Conclusions: Carrying a diagnosis of IPF, CPFE, fibrotic ILD, or non-fibrotic ILD with PH has an increased risk of mortality as compared to COPD with PH. Hemodynamic, PVR > 5 WU, 6 min walk test less than 150 m, as well as spirometric data including FEV1 < 30% and FVC < 40% predicted were independently associated with an increased risk of death. [ABSTRACT FROM AUTHOR]

Granulomatous tubulointerstitial nephritis (GTIN) attributed to early onset sarcoidosis is an ultrarare finding in an allograft kidney biopsy. We present the case of a young man with allograft dysfunction who had GTIN upon biopsy. We performed a thorough case review based on recovered records from early childhood and reassessed genetic testing results. We revised his underlying diagnosis from cryopyrin-associated periodic syndrome to early-onset sarcoidosis with wild-type NOD2 and established a rationale to use the interleukin-6 (IL-6) receptor blocker tocilizumab (TCZ). This suppressed his inflammatory disease and stabilised kidney function. We performed a literature review related to the emerging role of IL-6 pathway blockade in kidney transplantation. We identified 18 reports with 417 unique patients treated with TCZ for indications including HLA-desensitisation, transplant immunosuppression induction, treatment of chronic antibody-mediated rejection, and treatment of subclinical rejection. Both TCZ and the direct IL-6 inhibitor clazakizumab are being studied in ongoing randomised control trials. [ABSTRACT FROM AUTHOR]

PurposeMethodsResultsConclusionUveitis and scleritis may be caused by local or systemic infection, or associated with noninfectious systemic inflammatory autoimmune disease. This study explored the all-cause mortality following an individual’s first presentation with uveitis/scleritis.A cross-sectional study was conducted on all uveitis/scleritis patients diagnosed by uveitis specialists and treated in a single tertiary referral center in New Zealand between 2006 and 2020(15y). Masquerade syndromes including intraocular lymphoma were excluded. Outcome measures: demographics, etiology of uveitis/scleritis, anatomical location and all-cause mortality.2723 subjects were identified. Median age of onset of uveitis/scleritis was 44.9 years (Range:1.5–99.5 years). 49.6% were female. Median follow-up from diagnosis of uveitis/scleritis was 8.0 years (IQR 4.1–11.6 years) with a total follow-up of 24 443.3 subject-years. The most frequent diagnosis was idiopathic disease (30.9%), HLA-B27-positive uveitis (20.0%), and sarcoidosis (4.7%). Infectious etiologies (24.1%) were most commonly from herpes zoster virus (9.3%) and toxoplasmosis (4.3%). The age-adjusted mortality rate was higher in subjects with idiopathic disease, sarcoidosis, Fuchs’ uveitis syndrome, granulomatosis with polyangiitis/ANCA-associated vasculitis, toxoplasmosis, and herpes zoster virus, when compared to HLA-B27-positive uveitis. Hazard of mortality peaked in the first seven years following diagnosis, then subsequently declined. Patients with uveitis/scleritis had a significantly higher rate of mortality compared to the general New Zealand population (IRR 1.656 p = 0.017).Infectious etiologies of uveitis/scleritis in this cohort were high when compared to other developed nations, attributable to data from a tertiary referral center treating inpatients. Potential shared inflammatory mechanisms in the eye and other organs can lead to concurrent non-ocular disease requiring systemic treatment, impacting an individual’s longevity. [ABSTRACT FROM AUTHOR]