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Central nervous system (CNS) tuberculosis is a postprimary form of tuberculosis with high mortality and morbidity rates, even with early diagnosis and treatment. Focal tuberculous cerebritis is extremely rare, typically occurring in patients without AIDS, and often associated with tuberculous meningitis. In endemic regions, it should be a primary consideration when encountering cerebral anomalies suggestive of granulomatous conditions. Its meningeal pseudo-tumor form poses a significant diagnostic challenge.We present the case of a 26-year-old man who arrived at the emergency room with cerebellar and pyramidal syndrome. Cerebral magnetic resonance imaging (MRI) revealed an expansive lesion in the right hemicerebellum with glove-finger edema and leptomeningeal thickening. Given the MRI findings and associated lung involvement, the diagnosis of tuberculosis was strongly suggested in our epidemiological context. Due to contraindications for lumbar puncture, high surgical risk, and strong clinical and radiological suspicion, antituberculous treatment was initiated in collaboration with neurologists and infectious disease specialists.The clinical and radiologic manifestations of CNS tuberculosis can mimic other infectious and noninfectious neurological conditions, as seen in our patient. Thus, familiarity with the imaging presentations of CNS tuberculosis among infectious disease specialists and radiologists is crucial for prompt and accurate diagnosis.

Granulomatosis with polyangiitis is an ANCA-positive vasculitis primarily affecting small to medium-sized vessels, typically involving the lungs and kidneys. This article presents a remarkable case of a patient with an unusual pyoderma gangrenosum-like ulcer on the face and granulomatosis with polyangiitis. A 28-year-old man, previously diagnosed with granulomatosis with polyangiitis presented at a dermatology clinic with a 6-month-old ulcer in the right preauricular region following a traumatic incident. A spindle biopsy revealed suppurative granulomatous dermatitis with secondary vascular damage, while cultures showed no signs of infection. The patient was subsequently treated with rituximab, leading to a positive clinical response. This case underscores the challenge of diagnosing this rare manifestation of granulomatosis with polyangiitis and suggests that rituximab could be a valuable treatment option.

Sarcoidosis is a multi-system granulomatosis of unknown etiology, defined by the presence of epithelioid and gigantocellular granulomas, without caseous necrosis. Ocular sarcoidosis manifests mainly as bilateral granulomatous anterior uveitis. Occlusion of the central retinal vein in sarcoidosis is a rare manifestation, which is the particularity of our observation.

La réaction paradoxale tuberculeuse est définie par l'aggravation de lésions tuberculeuses présentes au diagnostic ou l'apparition de nouvelles lésions en cours de traitement antituberculeux, en l'absence d'autre cause spécifique surajoutée. Sa prévalence est estimée entre 5 à 30 % des cas, et dépend des sites infectés et du terrain. Le diagnostic de réaction paradoxale est un diagnostic d'élimination, et il suppose notamment d'avoir exclu un échec thérapeutique, notamment lié à une mauvaise observance ou à la présence d'antibiorésistance. L'atteinte neurologique fait la gravité de cette manifestation clinique. Malgré l'importance clinique de la réaction paradoxale tuberculeuse, sa physiopathologie reste mal comprise et sa prise en charge peu consensuelle. Celle-ci repose essentiellement sur la corticothérapie. La place des anti-TNF, actuellement proposée en cas de corticodépendance ou corticorésistance, reste à définir. Paradoxical tuberculosis reaction is defined as the aggravation of lesions present at diagnosis or the development of new lesions under anti-tuberculosis treatment, after exclusion of other alternate causes. It affects 5 to 30% of tuberculosis patients, with a variable prevalence depending on the site of infection and the clinical background. The diagnosis of paradoxical reaction is one of elimination, and requires having ruled out therapeutic failure, notably linked to poor compliance and/or to the presence of mycobacterial antibiotic resistance. The severity of paradoxical tuberculosis reaction lies in its neurological impairment. Despite its clinical importance, the mechanisms involved remain poorly understood and its management is not consensual. Corticosteroids are the cornerstone in the medical management. The role of anti-TNF agents, currently proposed in cases of corticodependence or corticoresistance, remains to be properly defined. [ABSTRACT FROM AUTHOR]

Granulomatous disease is a serious complication of common variable immunodeficiency (CVID-GD) that occurs in 8–22% of these patients and can mimic sarcoidosis, with which it shares certain clinical, biological, and radiological features. However, few studies to date have compared the two pathologies immunologically and histologically. Therefore, we analyzed the immunological-histological findings for different tissue samples from ten patients with CVID-GD and compared them to those of biopsy-proven sarcoidosis. Specifically, we wanted to know whether or not the signaling abnormalities observed in sarcoidosis granulomas are also present in CVID-GD. Morphological differences were found between CVID-GD histology and classical sarcoidosis: mainly, the former's notable lymphoid hyperplasia associated with granulomas not observed in the latter. All CVID-GD involved organs contained several follicular helper-T (TFH) cells within the granulomatosis, while those cells were inconstantly and more weakly expressed in sarcoidosis. Moreover, CVID and sarcoidosis granulomas expressed the phosphorylated-signal transducer and activator of transcription (pSTAT)1 and pSTAT3 factors, regardless of the organ studied and without any significant difference between entities. Our results suggest that the macrophage-activation mechanism in CVID resembles that of sarcoidosis, thereby suggesting that Janus kinase (JAK)-STAT–pathway blockade might be useful in currently difficult-to-treat CVID-GD. [ABSTRACT FROM AUTHOR]

Tuberculosis (TB) remains a significant global health concern and kills millions of people every year. While TB can affect any organ in the body, breast TB is relatively uncommon. This study presents a comprehensive review of literature spanning 23 years, with a focus on cases of breast TB in Iran. Among the 96 cases found, the majority (89.6%) fell within the age range of 20–60, with a striking prevalence among women (98.9%). Common symptoms included pain and palpable mass, each presenting in approximately 60.4% of cases. Notably, only a quarter of patients had a confirmed history of exposure to a known TB case. Left breast involvement was more prevalent (58.3%), with ipsilateral lymph node enlargement observed in 40.6% of cases. Given the clinical presentation of breast TB, which often leads to misdiagnosis, a significant proportion of cases (68.7%) were diagnosed through excisional biopsy. Following a standard 6-month regimen of anti-TB drugs, relapse occurred in only 4.2% of cases. This study highlights the need for heightened awareness and vigilance in diagnosing breast TB, especially in regions with a high burden. Although breast TB poses diagnostic challenges, with prompt identification and treatment, the prognosis is generally favorable, with a low incidence of relapse.

Introduction: We occasionally encounter irregular marginated masses discovered incidentally in young individuals. In most cases, further investigations are conducted to assess the presence of a primary malignancy, as these masses often raise suspicions of malignancy. However, rare exceptional cases leave us perplexed. Granulomas arising from common lung infections and those induced by foreign substances can often pose challenge in distinguishing them from lung cancer. Therefore, we aimed to present a case of multiple pulmonary granulomatosis following cosmetic procedure. Case Presentation: A 55-year-old woman visited the hospital after an incidental discovery of an abnormal chest radiograph during a routine health check-up. Subsequent computed tomography (CT) scans showed worrisome lung nodules, leading to biopsies and positron emission tomography CT scans. Histological examination of the biopsied specimens revealed a chronic inflammatory reaction surrounded by multinucleated foreign body giant cells. Upon sharing the biopsy results with the patient and conducting additional history-taking, she had undergone various cosmetic procedures (botox injection, dermal filler treatments, and thread lifts) around the face and neck, approximately 5–6 months ago. It was hypothesized that these cosmetic materials might have led to the observed pulmonary granulomatosis. After 3 months of conservative care, a follow-up CT showed no change in the lesions. Conclusion: We present this case to underscore the importance of considering pulmonary foreign body granulomatosis as a potential differential diagnosis, especially when it closely resembles lung cancer, particularly following cosmetic injections. [ABSTRACT FROM AUTHOR]

IntroductionAsthma associated with eosinophilic granulomatosis with polyangiitis (EGPA) is often severe and corticosteroid-dependent, leading to significant morbidity. Mepolizumab and benralizumab are humanized monoclonal antibodies targeting interleukin 5 (IL-5) and its receptor, respectively. They have been shown to be effective in steroid-sparing in patients with severe eosinophilic asthma.ObjectiveOur aim was to evaluate the efficacy and safety of mepolizumab and benralizumab prescribed for severe asthma in patients with EGPA under “real-world” conditions.MethodsThis was a retrospective analysis of patients with EGPA and persistent asthma who received either mepolizumab 100 or 300 mg administered every 4 weeks, or benralizumab 30 mg administered every 4 weeks for the initial 3 injections and followed by an injection every 8 weeks thereafter, whilst combined with oral glucocorticoids. The follow-up every 6 ± 3 months included an assessment of clinical manifestations, pulmonary function tests and eosinophil cell count. The primary outcome was the proportion of patients at 12 months receiving a daily oral dose of prednisone or equivalent of 4 mg or less with a BVAS of 0.ResultsTwenty-six patients were included. After 12 months of treatment with mepolizumab or benralizumab, 32% of patients met the primary outcome and were receiving less than 4 mg of prednisone per day with a BVAS of 0. The median dose of prednisone was 10 mg per day at baseline, 9 mg at 6 months, and 5 mg at 12 months (p ≤ 0.01). At 12 months, 23% of patients were weaned off corticosteroids, while an increase or no change in dose was observed in 27% of patients. The median eosinophil count was significantly reduced from 365 cells/mm3 to 55 cells/mm3 at 6 months and 70 cells/mm3 at 12 months, respectively. No significant change was observed in FEV1. After 12 months of treatment, 14% of patients had had an average of 1 exacerbation of asthma, compared with 52% of patients before baseline. The tolerability profile was favorable.ConclusionIn this real-world study in patients with severe asthma and a history of EGPA asthma, mepolizumab and benralizumab had a significant steroid-sparing effect and reduced asthma exacerbation, but no significant effect on lung function.

The most common cause of bilateral pulmonary cavities with constitutional symptoms in an endemic country like India is pulmonary tuberculosis (PTB). Pulmonary manifestations in systemic vasculitis are very diverse, ranging from nodules, consolidation, and cavity. Wegener’s granulomatosis (WG) is a systemic disease commonly affecting the lungs. Diffuse alveolar hemorrhage (DAH) is a life-threatening and relatively rare pulmonary manifestation of WG. Here, we report a case of a young male diagnosed with WG mimicking PTB with hypoxic respiratory failure who responded to aggressive treatment with cyclophosphamide, high-dose steroids and high-flow nasal cannula support (HFNC).

Abstract Background Ataxia-telangiectasia (A-T) is a rare autosomal recessive DNA repair disorder, characterized by progressive cerebellar degeneration, telangiectasia, immunodeficiency, recurrent sinopulmonary infections, radiation sensitivity, premature aging and predisposition to cancer. Although the association with autoimmune and chronic inflammatory conditions such as vitiligo, thrombocytopenia and arthritis has occasionally been reported, an onset with articular involvement at presentation is rare. Case presentation We herein report the case of a 7-year-old Caucasian girl who was admitted to the Rheumatology Department with a history of febrile chough and polyarthritis which led initially to the suspicion of an autoinflammatory disease. She had overt polyarthritis with knees deformities and presented with severe pneumonia. A chest Computed Tomography (CT) scan showed bilateral bronchiectasis, parenchymal consolidation and interstitial lung disease; rheumatoid factor and type I interferon signature resulted negative, therefore excluding COatomer Protein subunit Alpha (COPA) syndrome. A diagnosis of sarcoidosis had been suspected based on histological evidence of granulomatous liver inflammation, but ruled out after detecting normal angiotensin converting enzyme and chitotriosidase blood levels. Based on her past medical history characterized by at least six episodes of pneumonia in the previous 4 years, immunological phenotyping was performed. This showed complete IgA and IgE deficiency with defective antigen-specific antibodies to Pneumococcal, Tetanus toxin and Hemophilus Influenzae B vaccines. Additionally, low numbers of B cells and recent thymic emigrants (RTE) were found (CD4Ra 1.4%), along with a low CD4+/CD8 + T cells ratio (

Sarcoidosis is a systemic granulomatous disease of unknown etiology. Involvement of the lacrimal gland during sarcoidosis is a rare manifestation, most often occurring during multisystem involvement. Observation: We report the case of a 58-year-old woman who presented with a palpebral swelling of the right eye that had been evolving for four months, without any other associated signs. The diagnosis of a localized sarcoidosis dacryoadenitis was retained in view of the demonstration of a granulomatous lesion by histology and an elevated conversion enzyme in the absence of arguments for another granulomatous pathology. The patient was put on corticosteroid therapy with a good clinical evolution. Conclusion: Our case is exceptional because of its rare and inaugural location of the disease. Nevertheless, it is imperative to look for associated systemic manifestations, being sometimes more serious. [ABSTRACT FROM AUTHOR]

Background: Ataxia-telangiectasia (A-T) is a rare autosomal recessive DNA repair disorder, characterized by progressive cerebellar degeneration, telangiectasia, immunodeficiency, recurrent sinopulmonary infections, radiation sensitivity, premature aging and predisposition to cancer. Although the association with autoimmune and chronic inflammatory conditions such as vitiligo, thrombocytopenia and arthritis has occasionally been reported, an onset with articular involvement at presentation is rare. Case presentation: We herein report the case of a 7-year-old Caucasian girl who was admitted to the Rheumatology Department with a history of febrile chough and polyarthritis which led initially to the suspicion of an autoinflammatory disease. She had overt polyarthritis with knees deformities and presented with severe pneumonia. A chest Computed Tomography (CT) scan showed bilateral bronchiectasis, parenchymal consolidation and interstitial lung disease; rheumatoid factor and type I interferon signature resulted negative, therefore excluding COatomer Protein subunit Alpha (COPA) syndrome. A diagnosis of sarcoidosis had been suspected based on histological evidence of granulomatous liver inflammation, but ruled out after detecting normal angiotensin converting enzyme and chitotriosidase blood levels. Based on her past medical history characterized by at least six episodes of pneumonia in the previous 4 years, immunological phenotyping was performed. This showed complete IgA and IgE deficiency with defective antigen-specific antibodies to Pneumococcal, Tetanus toxin and Hemophilus Influenzae B vaccines. Additionally, low numbers of B cells and recent thymic emigrants (RTE) were found (CD4Ra 1.4%), along with a low CD4+/CD8 + T cells ratio (< 1). Finally, based on gait disturbances (wobbly wide-based walking), serum alfa-fetoprotein was dosed, which resulted increased at 276 ng/ml (normal value < 7 ng/ml). A diagnosis of Ataxia-Telangiectasia was made, strengthened by the presence of bulbar telangiectasia, and then confirmed by Whole Exome Sequencing (WES). Conclusions: Although rare, A-T should always be ruled out in case of pulmonary bronchiectasis and gait disturbances even in the absence of bulbar or skin telangiectasia. Autoimmune and granulomatous disorders must to be considered as differential diagnosis. [ABSTRACT FROM AUTHOR]

Copyright of Studia Pneumologica et Phthiseologica is the property of TRIOS, spol. sr.o. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Chronic rhinosinusitis (CRS) with (CRSwNP) or without (CRSsNP) nasal polyps is a prevalent and heterogeneous disorder existing as a spectrum of clinical conditions with complex underlying pathomechanisms. CRS comprises a broad syndrome characterized by multiple immunological features involving complex interactions between the genes, the microbiome, host- and microbiota-derived exosomes, the epithelial barrier, and environmental and micromilieu exposures. The main pathophysiological feature is an epithelial barrier disruption, accompanied by microbiome alterations and unpredictable and multifactorial immunologic overreactions. Extrinsic pathogens and irritants interact with multiple epithelial receptors, which show distinct expression patterns, activate numerous signaling pathways, and lead to diverse antipathogen responses. CRSsNP is mainly characterized by fibrosis and mild inflammation and is often associated with Th1 or Th17 immunological profiles. CRSwNP appears to be associated with moderate or severe type 2 (T2) or Th2 eosinophilic inflammation. The diagnosis is based on clinical, endoscopic, and imaging findings. Possible CRS biomarkers from the peripheral blood, nasal secretions, tissue biopsies, and nasally exhaled air are studied to subgroup different CRS endotypes. The primary goal of CRS management is to maintain clinical control by nasal douching with isotonic or hypertonic saline solutions, administration of nasal and systemic steroids, antibiotics, biologic agents, or, in persistent and more severe cases, appropriate surgical procedures. [ABSTRACT FROM AUTHOR]

We studied granuloma formation and its outcomes in BCG-induced granulomatosis in the liver of mice of different age periods treated with oxidized dextran. Newborn C57BL/6 mice were intraperitoneally injected with BCG vaccine on the first day of life (group 1) or BCG vaccine solution on first day of life and oxidized dextran on the second day of life (group 2). Analysis was carried out on 3, 5, 10, 28, and 56 days of life. After injection of BCG vaccine, granulomas in the liver appeared starting from the day 28. In mice treated with oxidized dextran, granulomas on day 28 were smaller and less numerous than in group 1 animals. In BCG granulomatosis, fibroplastic processes in the liver develop mainly at the site of granulomas. Injection of oxidized dextran under conditions of BCG granulomatosis reduced the manifestations of fibrosis in the liver. [ABSTRACT FROM AUTHOR]

Les maladies de système (connectivites, granulomatoses) peuvent s'associer de façon non rare à la survenue de neuropathies périphériques. Le diagnostic étiologique peut être complexe si la neuropathie inaugure la maladie. Il nécessite alors une étroite collaboration entre neurologues et internistes. À l'inverse, lorsque la maladie de système est déjà connue, la question principale reste celle de son imputabilité pour la neuropathie. Quelle que soit la situation, le diagnostic positif de la neuropathie se base sur une enquête électroclinique systématique et rigoureuse, précisant la topographie, l'évolutivité et le mécanisme de l'atteinte nerveuse. Certains examens d'imagerie, tel que l'IRM nerveuse et/ou plexique, ou parfois d'autres examens plus invasifs (biopsie cutanée ou neuromusculaire) permettent de préciser la topographie et le mécanisme lésionnel. L'imputabilité de la neuropathie au cours d'une maladie de système connue va principalement dépendre de son pattern électroclinique, dont les diagnostics alternatifs dépendent. Lors d'une neuropathie inaugurale, plusieurs éléments vont permettre d'orienter le diagnostic, comprenant le terrain sous-jacent (sujet jeune), d'éventuelles manifestations systémiques associées au second plan, (arthralgies inflammatoires, polyadénopathies), des résultats d'examens biologiques de première intention (lymphopénie, hypergammaglobulinémie, hypocomplémentémie), des auto-anticorps (antinucléaires, anti-ADN natifs, anti-SSA/B) et parfois des examens invasifs (biopsie neuromusculaire). Systemic diseases (connective disease, granulomatosis) may be associated with peripheral neuropathies. The diagnosis can be complex when the neuropathy is the presenting manifestation of the disease, requiring close collaboration between neurologists and internists. Conversely, when the systemic disease is already known, the main question remaining is its imputability in the neuropathy. Regardless of the situation, the positive diagnosis of neuropathy is based on a systematic and rigorous electro-clinical investigation, specifying the topography, the evolution and the mechanism of the nerve damage. Certain imaging examinations, such as nerve and/or plexus MRI, or other more invasive examinations (skin biopsy, neuromuscular biopsy) enable to specify the topography and the mechanism of the injury. The imputability of the neuropathy in the course of a known systemic disease is based mainly on its electro-clinical pattern, on which the alternatives diagnoses depend. In the case of an inaugural neuropathy, a set of arguments orients the diagnosis, including the underlying terrain (young subject), possible associated systemic manifestations (inflammatory arthralgias, polyadenopathy), results of first-line laboratory tests (lymphopenia, hyper-gammaglobulinemia, hypocomplementemia), autoantibodies (antinuclear, anti-native DNA, anti-SSA/B) and sometimes invasive examinations (neuromuscular biopsy). [ABSTRACT FROM AUTHOR]

Waldenström macroglobulinemia (WM) is a slowly progressive hematologic malignancy that usually responds rapidly to treatment. Being a lymphoplasmacytoid neoplasm, it is associated with a monoclonal IgM component, which may be associated with multiple manifestations and symptoms. We report the case of a 77-year-old woman diagnosed with WM following the development of severe and sudden pancytopenia associated with a cold agglutinin syndrome. In order to treat the WM and the underlying hemolysis, treatment with rituximab, corticosteroids and cyclophosphamide was started. Despite the improvement in hemolysis parameters, pancytopenia persisted, and we started a second line with ibrutinib. During treatment the patient developed an uncommon invasive fungal infection (IFI) with bone marrow granulomatosis and myelofibrosis. This case shows an unusual clinical course with a poor hematopoietic response to treatment and a large number of intercurrent complications. [ABSTRACT FROM AUTHOR]

Case reportWe present a case of a 48-year-old woman with 27 months of exposure to aluminum dust and silica owing to polishing processing. The patient was admitted to our hospital with intermittent cough and expectoration. Chest high-resolution computed tomography showed diffuse ill-defined centrilobular nodules and patchy ground-glass opacities in bilateral lungs. A video-assisted thoracoscopic surgery biopsy demonstrated multiple isolated and confluent granulomas in an otherwise normal parenchyma without malignancy or signs of infection. Elemental analysis was performed on the grinding wheel powder in the workplace using an X-ray fluorescence spectrometric analyzer, showing 72.7% of Al2O3 and 22.8% of SiO2 as raw materials. She was diagnosed with aluminum-associated sarcoid-like granulomatous lung disease, rather than sarcoidosis, according to occupational exposure by a multidisciplinary panel.ConclusionOccupational aluminum dust exposure may induce pulmonary sarcoid-like granulomatosis recognized by a multidisciplinary diagnostic panel.

Cardiac sarcoidosis typically involves the myocardium. Pericardial effusion is uncommon, and symptomatic pericardial disease is even more infrequent. We report the case of a patient presenting with pericarditis as the first manifestation of sarcoidosis. A 50-year-old previously healthy man presented with chest pain and dyspnoea. The electrocardiogram confirmed the diagnosis of pericarditis. Computed tomography of the thorax showed pulmonary infiltrates with mediastinal and hilar adenopathies. Histological analysis of a lymph node biopsy was consistent with sarcoidosis. There was no evidence of myocardial involvement on Magnetic Resonance Imaging (MRI). We reviewed the available English literature and identified 31 cases with sarcoidosis and pericardial involvement. The majority of cases presented as pericardial effusion, which was often the first clinical manifestation of the disease. Pathological diagnosis usually occurs at extra-cardiac locations. Myocardial involvement, an important cause of morbidity and mortality, was found in 25.8% (8/31) of cases. Sarcoidosis should be considered in the differential diagnosis of patients presenting with pericardial disease. The optimal treatment regimen and long-term outcome remain largely unknown. Research in cardiac sarcoidosis should include pericardial disease as a separate manifestation in order to improve the management of this rare but likely underdiagnosed condition. [ABSTRACT FROM AUTHOR]

The pediatric vasculitides are a relatively uncommon and heterogeneous group of disorders characterized by vessel inflammation, often with cardiothoracic involvement. Diagnosis and monitoring are often clinically challenging because of the nonspecific symptoms and laboratory markers. Thus, imaging has assumed increasing importance for early detection of disease activity, extent and complications as well as long-term monitoring pre- and post-treatment. Herein, we review the major pediatric vasculitides with frequent chest manifestations, including Takayasu arteritis, Kawasaki disease, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, Behçet disease and potential mimics. We highlight key clinical features and management considerations, emphasizing the central role of imaging. [ABSTRACT FROM AUTHOR]

Isolated laryngeal tuberculosis is rare and sometimes difficult to diagnose. It is the most common cause of laryngeal granuloma. We here report the case of a 58-year-old man, with no particular past medical history, hospitalized due to paroxysmal laryngeal dyspnea, dysphagia to solid foods and dysphonia evolving for 6 months without other associated signs. Laryngoscopic examination showed polyploid formation masking the glottic floor. Histological examination revealed epithelioid and gigantocellular granuloma, without caseous necrosis. Direct microscopic examination and culture were negative. The diagnosis of isolated laryngeal tuberculosis was made based on the endemicity in our country and the absence of other arguments in favor of another type of granulomatosis. Anti-tuberculosis therapy, combined with oral corticosteroids, was indicated based on the presence of severe upper airway edema and symptoms were resolved after 40 days of treatment. [ABSTRACT FROM AUTHOR]

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Immunological checkpoint inhibitors are now part of the oncological therapeutic arsenal in many solid cancers and malignant blood diseases, at the cost of immuno-mediated toxicities, of which dermatological disorders are among the most frequent. The most common, following treatment with anti-PD1 or anti-CTLA4, are maculopapular erythema, pruritus, vitiligo, or lichenoid lesions, but other more atypical conditions may lead to the internist being called upon. Here, we present a case series of these less common dermatological manifestations including fasciitis, dermatomyositis, scleroderma, granulomatosis and immune-induced vasculitis. Some manifestations appear similar to the primary forms or seem to correspond to paraneoplastic syndromes, but some diagnostic and therapeutic particularities are specific to ICI toxicity that the internist must be aware of., (Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Anti-neutrophil cytoplasmic autoantibody (ANCA) vasculitis has diverse patterns of injury including microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Necrotizing and crescentic glomerulonephritis (NCGN) occurs in all syndromes and as renal limited vasculitis (RLV). Single-dose intravenous ANCA IgG-specific for mouse myeloperoxidase (MPO) causes RLV in mice. Although multiple mouse models have elucidated ANCA-IgG induced necrotizing and crescentic glomerulonephritis (NCGN), pathogenesis of ANCA-induced granulomatosis and vasculitis outside the kidney has not been clarified. To investigate this, we used intravenous MPO-ANCA IgG in the same strain of mice to induce different patterns of lung disease mirroring patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Repeated intravenous MPO-ANCA IgG induced GPA with NCGN, lung capillaritis, arteritis and granulomatosis. Lung leukocyte phenotypes were evaluated by immunohistochemical image analysis and by flow cytometry. ANCA lung capillaritis and microabscesses began within one day and evolved into granulomas in under seven days. Influenza plus single-dose MPO-ANCA IgG induced MPA with NCGN, lung capillaritis and arteritis, but no granulomatosis. Allergic airway disease caused by house dust mites or ovalbumin plus single-dose intravenous MPO-ANCA IgG induced EGPA with eosinophilic bronchiolitis, NCGN, capillaritis, arteritis, and granulomatosis. Thus, our study shows that the occurrence and pattern of lung lesions are determined by the same ANCA IgG accompanied by different synergistic immune factors., (Copyright © 2024 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Background: The diagnosis and management of neurosarcoidosis (NS) in pediatric patients remain challenging, with limited case documentation to guide clinicians. Most existing reports focus on initial presentations. This study aimed to outline the clinical features, management, and medium-term outcomes of pediatric NS METHODS: In this retrospective, multicentric, observational study, we collected data from pediatric patients followed in French pediatric rheumatology centers with a diagnosis of NS between January 2001 and June 2023., Results: We identified 11 patients diagnosed with NS, comprising eight girls and three boys. The mean age at diagnosis of sarcoidosis was 10 (5 to 15) years, and the mean age of diagnosis of NS was 11.5 (5 to 17) years. Predominant neurological symptoms included headache (nine of 11 patients), papilledema (6 of 11 patients), facial palsy (two patients), seizures (one patient), and motor deficit (two patients). Nine of 11 patients had eye involvement, which consisted of granulomatous and bilateral uveitis. All patients exhibited meningitis, with cerebrospinal fluid white blood cell counts ranging from 6 to 70 cells/mm 3 . Six individuals presented neurological abnormalities on imaging, detailed in this study. Treatment primarily involved corticosteroids, methotrexate, and tumor necrosis factor alpha (TNF-alpha) inhibitors. Biologics targeting TNF-alpha were necessary to achieve remission in eight of 11 patients. In two patients who did not receive this treatment initially, it was required later in the course of evolution., Conclusions: This study enhances understanding of the clinical course of pediatric NS and supports the early use of TNF-alpha biologics for improved management in affected children., Competing Interests: Declaration of competing interest We declare no competing interest., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Neurocryptococcosis is a severe neurological complication of Cryptococcus neoformans infections, primarily affecting immunocompromised individuals. This report describes the case of a 53-year-old man with no known medical history who experienced severe headaches and vomiting while on a business trip to Pakistan. He was given preventive antibiotic therapy, followed by a combination of sulfamethoxazole and trimethoprim for suspected toxoplasmosis. The patient's condition initially improved and he was discharged from the hospital, but later experienced a recurrence of symptoms and sought emergency care. The diagnosis of neurocryptococcosis was confirmed through various biological tests, including flow cytometry. Treatment with Amphotericin B and 5-Fluorocytosine was initiated. Further testing revealed significant CD4+ T-cell lymphopenia, which was attributed to sarcoidosis-like systemic granulomatosis. This case presents an atypical clinical manifestation, with the abrupt onset of an opportunistic infection in a patient without any known immunosuppression.

Background: Idiopathic granulomatous mastitis (IGM) is a rare relapsing benign, chronic inflammatory breast disease characterized by infiltration of inflammatory cells including multinucleated giant cells. Recently, it has been suggested that infection with Corynebacterium species (spp.) may be involved in the onset of the disease. Therefore, the aim of this article is to summarize the previous IGM reports related to Corynebacterium spp. Methods: I used the terms "granulomatous mastitis Corynebacterium" in PubMed and "granulomatous mastitis Corynebacterium" with Japanese in Google Scholar, which resulted in 63 English articles and 71 Japanese articles. I read all the abstracts and summarized the recent articles with Corynebacterium in the title. Results: In 16 English articles and 4 Japanese ones, the most common Corynebacterium spp. was Corynebacterium kroppenstedtii, followed by Corynebacterium tuberculostearium. Conclusion: Corynebacterium infection is widely detected with IGM. In addition to bacterial culture, real-time polymerase chain reaction and formalin-fixed, paraffinembedded biopsy specimens' analysis can be used to detect Corynebacterium spp. [ABSTRACT FROM AUTHOR]

Simple Summary: Granulomatosis is commonly observed in patients receiving immune checkpoint inhibitors (ICIs) for melanoma and other cancers. Recognizing this reaction early is critical to avoid confusing it with cancer progression. Our objectives were to characterize instances of granulomatosis triggered by immunotherapy, to question their relationship with sarcoidosis, to explore their potential pathophysiological mechanisms and to address the possibility of an association between such events and response to immunotherapy. Our work is based on a retrospective study of 18 cases and on a thorough review of the literature of melanoma patients developing ICI-associated granulomatosis. After analyzing the clinical, histopathological and biological results, we propose that immuno-induced granulomatosis corresponds to an experimental sarcoidosis and should be considered as an ICI-associated effect rather than a severe immune-related event. Indeed, ICI-associated granulomatosis seems to be associated with a good ICI response and usually does not require ICI interruption or symptomatic treatment. We aimed to review the clinical and biological presentation of granulomatosis associated with immune-checkpoint inhibitors (ICI) in patients with melanoma and to explore its association with classical sarcoidosis as well as with cancer response to ICI. To this end, a retrospective study on 18 melanoma patients with histologically proven ICI-induced granulomatosis over a 12-year period in a single center, as well as on 67 similar cases reported in the literature, was conducted. Results indicate ICI-induced granulomatosis is an early side effect (median time to onset: 2 months). Its clinical presentation, with predominant (90%) thoracic involvement, histopathological appearance and supposed underlying biology (involving the mTOR pathway in immune cells, Th17 polarization and TReg dysfunction) are indistinguishable from those of sarcoidosis. Moreover, it appears to be associated with ICI benefit (>65% objective response rate). Evolution is generally favorable, and symptomatic steroid treatment and/or ICI discontinuation are rarely necessary. ICI-associated granulomatosis is critical to explore for several reasons. Practically, it is essential to differentiate it from cancer progression. Secondly, this "experimental" sarcoidosis brings new elements that may help to address sarcoidosis origin and pathophysiology. Its association with ICI efficacy must be confirmed on a larger scale but could have significant impacts on patient management and biomarker definition. [ABSTRACT FROM AUTHOR]

Inhalation of cosmetic talc can lead to pulmonary foreign-body granulomatosis, though fewer than 10 cases of inhaled cosmetic talc-related pulmonary granulomatosis have been reported in adults. We report the case of a 64-year-old man with diffuse, bilateral pulmonary nodules and ground glass opacities associated with chronic inhalation of cosmetic talc. Transbronchial biopsy showed peribronchiolar foreign-body granulomas. After cessation of talc exposure, the patient demonstrated clinical and radiographic improvement without the use of corticosteroids. This case demonstrates that a conservative approach with cessation of exposure alone, without the use of corticosteroids, can be an effective therapy in cosmetic talc-related pulmonary granulomatosis.

Waldenström macroglobulinemia (WM) is a slowly progressive hematologic malignancy that usually responds rapidly to treatment. Being a lymphoplasmacytoid neoplasm, it is associated with a monoclonal IgM component, which may be associated with multiple manifestations and symptoms. We report the case of a 77-year-old woman diagnosed with WM following the development of severe and sudden pancytopenia associated with a cold agglutinin syndrome. In order to treat the WM and the underlying hemolysis, treatment with rituximab, corticosteroids and cyclophosphamide was started. Despite the improvement in hemolysis parameters, pancytopenia persisted, and we started a second line with ibrutinib. During treatment the patient developed an uncommon invasive fungal infection (IFI) with bone marrow granulomatosis and myelofibrosis. This case shows an unusual clinical course with a poor hematopoietic response to treatment and a large number of intercurrent complications.

Heerfordt syndrome is a rare clinical form of sarcoidosis with a favorable evolutionary profile in the majority of cases. In its classical form, it associates uveitis, parotidomegaly, facial paralysis and fever. We report a case of multisystemic sarcoidosis type II revealed by a Heerfordt syndrome in a 51-year-old female patient. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA) is a multi-system disorder associated with ocular, renal, cardiac, pulmonary involvement. However cardiac involvement is very rare compared to other systems. We present a case of an unusual presentation of GPA associated with ocular involvement super imposed on pre-existing rheumatic valvular disease. The cardiac involvement manifested as a thick fibro elastic membrane encasing the aortic and mitral valve annuli making the valve replacements a real technical challenge. The patient needed permanent pacemaker implantation due to intermittent complete heart block that was present even preoperatively. The patient was started on steroids and is doing well until the last follow-up. This case highlights the importance of recognition of GPA in cardiac surgery and to be aware of such an entity when one encounters a thick whitish avascular fibro elastic membrane encasing cardiac valves and endocardium, obliterating the anatomical delineation of structures during surgery. [ABSTRACT FROM AUTHOR]

Abstract Background and aim Breast tuberculosis is a rare disease, even in endemic areas. The diagnosis can be challenging, as it can mimic breast cancer. We aim to report our experience and discuss diagnoses and management modalities. Results We encountered twelve cases of breast tuberculosis in our institution from 2004 to 2019. The average age of our Caucasian North African patients was 42 years old (22–63). The classic presentation was a breast lump found in half of the cases. On physical examination, we suspected breast carcinoma in seven patients. The average size of the tumors was 39 mm (15–80 mm). Nine patients had a mammogram. In five cases, there was a suspicious breast mass mimicking a malignant tumor with an average size of 33 mm (25–60 mm). A ultrasonography was performed in 6 cases and revealed a suspicious ill-circumscribed nodule in four patients with an average size of 37.5 mm (10–60 mm). Five patients had a lumpectomy, and seven women underwent drainage of the abscess and the biopsy of its hull. The association of epithelioid cell granulomas and caseous necrosis was mandatory for the histological diagnosis of tuberculosis. All of them had an antitubercular therapy. The median period of follow-up was of 43 months (3–156 months). One patient presented with a recurrent abscess of the breast. Conclusion Our study found that clinical examination and radiological imaging were not specific. Positive cultures for Koch bacillus or histological confirmation are mandatory for the diagnosis. A meta-analysis of the existing cases is needed.

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La maladie des griffes du chat due à Bartonella henselae avec atteinte osseuse est rare. Le diagnostic est difficile et la prise en charge demeure non codifiée. Nous rapportons un cas de bartonellose disséminée avec ostéomyélite multifocale et atteintes viscérales multiples chez une adulte immunocompétente. La confirmation diagnostique était apportée par la PCR sur adénopathie. Avec notre observation, 31 cas de bartonellose avec atteinte osseuse ont été rapportés dans la littérature. Le diagnostic repose sur l'association de l'interrogatoire, de la sérologie et de la PCR sur tissu. Le traitement antibiotique permet dans tous les cas la guérison. La maladie des griffes du chat dans sa forme systémique avec atteinte osseuse est un diagnostic rare et difficile pour le clinicien et une approche invasive est souvent nécessaire pour obtenir le diagnostic. Cat scratch disease caused by Bartonella henselae with bone involvement is a rare presentation. We report a case of disseminated bartonellosis with multifocal osteomyelitis and multiple visceral involvement in an immunocompetent adult. Diagnostic confirmation was obtained by PCR on lymphadenopathy. In addition to our observation, 31 cases of bartonellosis with bone involvement were reported in the literature. Diagnosis is based on a combination of history, serology and PCR performed on tissue. The antibiotic treatment allows recovery in all cases. Cat scratch disease in its systemic form with bone involvement is a rare and difficult diagnosis for the clinician and an invasive approach is often required to obtain the diagnosis. [ABSTRACT FROM AUTHOR]

Autoinflammatory diseases (AIDs) are a heterogeneous group of disorders in which recurrent or continuous aseptic inflammation arises primarily through antigen-independent hyperactivation of the innate immune system. The skin is frequently involved with a wide variety of cutaneous manifestations, most of which are non-specific. Recognition of skin lesions in AIDs may sometimes provide clues for a correct diagnosis. In this review, the cutaneous involvements of >20 selected AIDs were summarized and organized into different categories based on their characteristic manifestations, such as urticarial dermatosis, neutrophilic dermatosis, granulomatosis, chilblain, lipodystrophy, and hyperkeratosis. With this classification scheme, cutaneous manifestations in AIDs could be more easily identified to facilitate diagnosis in clinical practice. [ABSTRACT FROM AUTHOR]

Granulomatous mastitis is a rare inflammatory disease of varying etiology. Tuberculosis and cystic neutrophilic granulomatous mastitis caused by Corynebacterium are the best-established infectious examples. Despite the increasing incidence of Rickettsia-related diseases worldwide, granulomatous inflammation of breast parenchyma caused by Rickettsia has not yet been reported. We present a unique case of bilateral granulomatous mastitis documented with mammography, magnetic resonance imaging and core-needle biopsy. The rickettsial etiology of the disease was proved with specific immunohistochemistry and confirmed with DNA extraction, PCR and serology. The lesions completely resolved after a full-course tetracycline treatment. This case report widens the knowledge about the possible clinical manifestations of Rickettsia infection and adds a new bacterium to the list of etiological factors causing granulomatous mastitis. [ABSTRACT FROM AUTHOR]

Abstract Introduction Granulomatosis with polyangiitis (GPA) is one of the forms of small vessel vasculitis. It is a rare condition that needs a high degree of suspicion to reach the diagnosis. It is one of the causes of diffuse parenchymal lung disease, with a very wide differential diagnosis. It is commonly misdiagnosed with malignant, granulomatous, and infectious lung diseases. Case presentation We report a case of a 31-year-old male who presented with productive cough, shortness of breath, hemoptysis, nasal obstruction, and epistaxis together with submandibular salivary gland swelling. Diagnosis of GPA was based on characteristic cavitary lung lesions, nasal and salivary gland involvement, pathological samples that revealed necrotizing granulomatous inflammation, characteristic positive Cytoplasmic- ANCA (C-ANCA), together with exclusion of malignancy and tuberculosis. Conclusion GPA is a rare condition. Salivary gland involvement should raise suspicion about GPA, in addition to other systemic manifestations.

Central nervous system (CNS) tuberculosis is a postprimary form of tuberculosis with high mortality and morbidity rates, even with early diagnosis and treatment. Focal tuberculous cerebritis is extremely rare, typically occurring in patients without AIDS, and often associated with tuberculous meningitis. In endemic regions, it should be a primary consideration when encountering cerebral anomalies suggestive of granulomatous conditions. Its meningeal pseudo-tumor form poses a significant diagnostic challenge. We present the case of a 26-year-old man who arrived at the emergency room with cerebellar and pyramidal syndrome. Cerebral magnetic resonance imaging (MRI) revealed an expansive lesion in the right hemicerebellum with glove-finger edema and leptomeningeal thickening. Given the MRI findings and associated lung involvement, the diagnosis of tuberculosis was strongly suggested in our epidemiological context. Due to contraindications for lumbar puncture, high surgical risk, and strong clinical and radiological suspicion, antituberculous treatment was initiated in collaboration with neurologists and infectious disease specialists. The clinical and radiologic manifestations of CNS tuberculosis can mimic other infectious and noninfectious neurological conditions, as seen in our patient. Thus, familiarity with the imaging presentations of CNS tuberculosis among infectious disease specialists and radiologists is crucial for prompt and accurate diagnosis., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Herein, we report a case of systemic sarcoidosis confirmed by a eyelid biopsy in a 47-year-old North African female with a past medical history of a bilateral granulomatous anterior and intermediate uveitis. She presented to the ophthalmological department for bilateral palpebral painful discomfort. An examination revealed the presence of bilateral, rounded, welllimited, subcutaneous, nodular lesions on the upper and inferior lid. A cutaneous biopsy confirmed the presence of a non-caseating granulomatous inflammation. The diagnosis of a definite ocular sarcoidosis was made and she was treated with local steroid ointment associated to hydroxychloroquine. A skin examination of the patient with granulomatous uveitis cannot be stressed enough. A biopsy allows us to make the diagnosis and to treat the patients appropriately. [ABSTRACT FROM AUTHOR]

Systematic granulomatosis is a chronic disease that affects the majority of farmed meagre (A. regius). Nutritional imbalances and overcrowding can increase the risk to suffer oxidative stress, and consequently, impact the incidence of granulomatosis. In order to evaluate this, juvenile meagre were fed five isolipidic (16.7%) and isoproteic (49.6%) fish meal and fish oil‐based feeds prepared by adding different levels of vitamin C, minerals (Mn, Zn, Se) with constant vitamin E and K (300 and 35 mg/kg respectively): Diet KEC (100 mg/kg C), Diet KEC+Mn/Zn/Se (100 mg/kg C, 40 mg/kg Mn, 200 mg/kg Zn, 1.5 mg/kg Se), Diet KECC (600 mg/kg C), Diet KECCC (1200 mg/kg C), Diet KECCCC (3200 mg/kg C). All diets were tested at 3.20 kg/m3, but diets KECC and KECCCC were also tested at 6.20 kg/m3. Growth performance was only affected by stocking density, being lower at high density. The percentage of fish with granulomas was significantly lower in fish fed with the highest dietary vitamin C contents (KECCC and KECCCC) at low density. TBARS content was correlated with the percentage of granulomas in the liver (R2 = 0.9439, y = 0.003x − 0.1242) denoting the involvement of an imbalance oxidative status in the appearance of granulomas. The present results show that high levels of vitamin C (1200–3200 mg/kg C) and low stock density (3.20 kg/m3) favours the growth of juvenile meagre, reducing the lipid peroxidation indicators and decreasing the incidence of granulomas, which confirms that this pathology is mostly triggered by the deficiency of antioxidant nutrients, particularly vitamin C. [ABSTRACT FROM AUTHOR]

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides include granulomatosis with polyangiitis (Wegener), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), necrotizing and crescentic glomerulonephritis that lacked well defined immunoglobulin deposit and drug-induced vasculitides. The five phenotypes are characterized by small vessel vasculitis, absence or paucity of vessel wall localization of immunoglobulin and complement, with or without granulomatous inflammation, most often in the lung, and the presence of circulating ANCA with specificity toward proteinase-3 or myeloperoxidase. The mechanism by which ANCAs cause vasculitis is unknown. Experimental and clinical evidence suggest that both PR-3 and MPO proteases are pathogenic. Traditionally, Fauci regimen of GPA consists of daily oral cyclophosphamide and glucorticoids. Other therapies include rituximab, plasmapheresis and mycophenolate. [ABSTRACT FROM AUTHOR]

Les pathologies parotidiennes peuvent être d'origine inflammatoire (sialite), métabolique ou tumorale. De nombreuses maladies systémiques au premier rang desquelles le syndrome de Sjögren et la sarcoïdose peuvent engendrer une hypertrophie parotidienne. Le diagnostic différentiel, parfois difficile, malgré les examens complémentaires les plus poussés (échographie, IRM, ponction cytologique à l'aiguille fine) sera infectieux, lithiasique ou tumoral. Parotid enlargement can be of inflammatory (sialitis), metabolic or tumoral. Many systemic diseases, mainly Sjögren's syndrome and sarcoidosis, can lead to parotid hypertrophy. The differential diagnosis, which is sometimes difficult, despite radiological investigations) (ultrasound, MRI, cytological fine needle puncture) will be infectious, lithiasis or tumor. [ABSTRACT FROM AUTHOR]