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Copyright of Respiratory Case Reports is the property of LookUs Scientific 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.)

Systemic Lupus Erythematosus (SLE) is a multifaceted, multisystem autoimmune disorder with diverse clinical expressions. While prevalence reports vary widely, pulmonary involvement accounts for significant morbidity and mortality in SLE. This comprehensive review explores the spectrum of pulmonary disease in SLE, including upper airway manifestations (e.g., laryngeal affection), lower airway conditions (e.g., bronchitis, bronchiolitis, bronchiectasis), parenchymal diseases (e.g., interstitial lung disease, acute lupus pneumonitis, diffuse alveolar hemorrhage), pleural diseases (e.g., serositis, shrinking lung syndrome), and vascular diseases (e.g., pulmonary arterial hypertension, pulmonary embolism, acute reversible hypoxemia syndrome). We discuss diagnostic modalities, treatment strategies, and prognosis for each pulmonary manifestation. With diagnostics remaining a challenge and with the absence of standardized treatment guidelines, we emphasize the need for evidence-based guidelines to optimize patient care and improve outcomes in this complex disease. [ABSTRACT FROM AUTHOR]

Background: Cryoglobulinemia with pulmonary involvement is rare, and its characteristics, radiological findings, and outcomes are still poorly understood. Methods: Ten patients with pulmonary involvement of 491 cryoglobulinemia patients at Peking Union Medical College Hospital were enrolled in this retrospective study. We analyzed the characteristics, radiological features and management of pulmonary involvement patients, and compared with those of non-pulmonary involvement with cryoglobulinemia. Results: The 10 patients with pulmonary involvement (2 males; median age, 53 years) included three patients with type I cryoglobulinemia and seven patients with mixed cryoglobulinemia. All of 10 patients were IgM isotype cryoglobulinemia. All type I patients were secondary to B-cell non-Hodgkin lymphoma. Four mixed patients were essential, and the remaining patients were secondary to infections (n = 2) and systemic lupus erythematosus (n = 1), respectively. Six patients had additional affected organs, including skin (60%), kidney (50%), peripheral nerves (30%), joints (20%), and heart (20%). The pulmonary symptoms included dyspnea (50%), dry cough (30%), chest tightness (30%), and hemoptysis (10%). Chest computed tomography (CT) showed diffuse ground-glass opacity (80%), nodules (40%), pleural effusions (30%), and reticulation (20%). Two patients experienced life-threatening diffuse alveolar hemorrhage. Five patients received corticosteroid-based regimens, and four received rituximab-based regimens. All patients on rituximab-based regimens achieved clinical remission. The estimated two-year overall survival (OS) was 40%. Patients with pulmonary involvement had significantly worse OS and progression-free survival than non-pulmonary involvement patients of cryoglobulinemia (P < 0.0001). Conclusions: A diagnosis of pulmonary involvement should be highly suspected for patients with cryoglobulinemia and chest CT-indicated infiltrates without other explanations. Patients with pulmonary involvement had a poor prognosis. Rituximab-based treatment may improve the outcome. [ABSTRACT FROM AUTHOR]

Copyright of Respiratory Case Reports is the property of LookUs Scientific 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.)

Copyright of Revista de Nefrología Diálisis y Trasplante is the property of Asociacion Regional de Dialisis y Transplantes Regionale 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.)

Background: Dysregulated long noncoding RNA (lncRNA) expression with increased apoptosis has been demonstrated in systemic lupus erythematosus (SLE) patients with alveolar hemorrhage (AH). SNHG16, a lncRNA, can enhance pulmonary inflammation by sponging microRNAs, and upregulate toll-like receptor 4 (TLR4) expression via stabilizing its mRNAs. TRAF6, a TLR4 downstream signal transducer, can induce autophagy and NETosis formation. In this study, we investigated whether SNHG16 could regulate TLR4-mediated autophagy and NETosis formation in SLE-associated AH. Methods: Expression of SNHG16, TLR4 and TRAF6 and cell death processes were examined in lung tissues and peripheral blood (PB) leukocytes from AH patients associated with SLE and other autoimmune diseases, and in the lungs and spleen from a pristane-induced C57BL/6 mouse AH model. SNHG16-overexpressed or -silenced alveolar and myelocytic cells were stimulated with lipopolysaccharide (LPS), a TLR4 agonist, for analyzing autophagy and NETosis, respectively. Pristane-injected mice received the intra-pulmonary delivery of lentivirus (LV)-SNHG16 for overexpression and prophylactic/therapeutic infusion of short hairpin RNA (shRNA) targeting SNHG16 to evaluate the effects on AH. Renal SNHG16 expression was also examined in lupus nephritis (LN) patients and a pristane-induced BALB/c mouse LN model. Results: Up-regulated SNHG16, TLR4 and TRAF6 expression with increased autophagy and NETosis was demonstrated in the SLE-AH lungs. In such patients, up-regulated SNHG16, TLR4 and TRAF6 expression was found in PB mononuclear cells with increased autophagy and in PB neutrophils with increased NETosis. There were up-regulated TLR4 expression and increased LPS-induced autophagy and NETosis in SNHG16-overexpressed cells, while down-regulated TLR4 expression and decreased LPS-induced autophagy and NETosis in SNHG16-silenced cells. Pristane-injected lung tissues had up-regulated SNHG16, TLR4/TRAF6 levels and increased in situ autophagy and NETosis formation. Intra-pulmonary LV-SNHG16 delivery enhanced AH through up-regulating TLR4/TRAF6 expression with increased cell death processes, while intra-pulmonary prophylactic and early therapeutic sh-SNHG16 delivery suppressed AH by down-regulating TLR4/TRAF6 expression with reduced such processes. In addition, there was decreased renal SNHG16 expression in LN patients and mice. Conclusions: Our results demonstrate that lncRNA SNHG16 regulates TLR4-mediated autophagy and NETosis formation in the human and mouse AH lungs, and provide a therapeutic potential of intra-pulmonary delivery of shRNA targeting SNHG16 in this SLE-related lethal manifestation. [ABSTRACT FROM AUTHOR]

Introduction: Systemic lupus erythematosus (SLE) is non-organ specific autoimmune disease with mainly skin, joint, and kidney involvement. SLE-related acute lung disease (ALD) is rare, poorly investigated and can lead to acute respiratory failure. We conducted a retrospective study aiming to describe clinical features, treatments and outcome of SLE-related APD. Methods: We retrospectively included all patients with SLE and ALD admitted from November 1996 and September 2018 to La Pitié-Salpêtrière Hospital, after exclusion of viral or bacterial lung infection, cardiac failure or any other alternate diagnosis. Results: During the time of the study, 14 patients with 16 episodes were admitted to our center: female 79%, mean age ± SD at admission 24 ± 11 years. ALD was inaugural of the SLE in 70% cases. SLE main organ involvement were: arthritis 93%, skin 79%, serositis 79%, hematological 79%, kidney 64%, neuropsychiatric 36% and cardiac 21%. 11 episodes required ICU admission for a median time of 8 days. Chest CT-scan revealed mostly basal consolidation and ground-glass opacities. When available, bronchoalveolar lavage mostly revealed a neutrophilic alveolitis with alveolar hemorrhage in 67% cases. Symptomatic respiratory treatments were: oxygen 81%, high-flow nasal canula oxygen 27%, non-invasive ventilation 36%, mechanical ventilation 64% and venovenous extracorporeal membrane oxygenation 18%. SLE-specific treatments were: corticosteroids 100%, cyclophosphamide 56% and plasma exchange 25%. All patients but one survived to ICU and hospital discharge. Two patients had a relapse of SLE-related ALD but none had interstitial lung disease during follow-up. Conclusion: Systemic lupus erythematosus-related acute respiratory failure is a severe event, mostly occurring at SLE onset, typical harboring a basal consolidation pattern on chest CT-scan and alveolar hemorrhage on BAL pathological examination. Mortality in our cohort is lower than previously reported but these results needs to be confirmed in further larger studies. [ABSTRACT FROM AUTHOR]

The study was conducted in the Department of Forensic Medicine, Government Medical College, Thiruvananthapuram. The period of the study was from January 2013 to June 2014. 50 cases were included among which 25 cases were the hanging cases that survived for varying periods before death and 25 cases were from the non-survived victims of hanging. Macroscopic and microscopic changes in the lungs, were compared to find out the changes resulting from hypoxic insult. The cases were studied in detail and are described below. [ABSTRACT FROM AUTHOR]

Objective : Diffuse alveolar hemorrhage (DAH) is a severe pulmonary complication of numerous diseases, including rheumatic conditions. We have conducted an observational study using inpatient data from the National Inpatient Sample to study the relationship of DAH with rheumatic conditions along with their descriptive characteristics. Methods: An observational study was conducted on hospitalizations in 2016–2018 with a principal diagnosis of DAH from the United States National Inpatient Sample database. A multivariate logistic regression analysis was performed to calculate adjusted odds ratios (ORadj) for risk factors of DAH. Results: A total of 5420 DAH hospitalizations were identified among 90 million hospitalizations. Mortality in this group was found to be 24.3%. Majority of patients admitted with DAH were white and male, with a mean age of 61.8 years and a mean LOS of 10.6 days. Multivariate analysis showed that multiple rheumatic diseases were associated with DAH, including anti-neutrophil cytoplasmic antibody–associated vasculitis (AAV) (ORadj 72.56) (95% C.I. 50.607–104.043), antiphospholipid antibody syndrome (APLS) (ORadj 6.51) (95% C.I. 3.734–11.366), eosinophilic granulomatosis with polyangiitis (EGPA) (ORadj 7.13) (95% C.I. 1.886–26.926), Goodpasture's (ORadj 30.58) (95% C.I. 16.360–57.176), rheumatoid arthritis (RA) (ORadj 1.60) (95% C.I. 1.158–2.212), sarcoidosis (ORadj 3.99) (95% C.I. 2.300–6.926), and systemic lupus (SLE) (ORadj 5.82) (95% C.I. 3.993–8.481). Conclusion: Although DAH is a relatively rare entity, it carries a very high mortality. Multiple rheumatic diseases were associated with DAH hospitalizations including AAV, APLS, EGPA, Goodpasture's, RA, sarcoidosis, and SLE. Key points • It is known that DAH carries a high morbidity and mortality based on prior literature. However, large datasets on the association of rheumatic diseases with DAH are lacking • This study identifies the descriptive characteristics of patients admitted to the hospital with DAH • This study also identifies the strength of association of rheumatic diseases with DAH [ABSTRACT FROM AUTHOR]

Cardiac etiologies of hemoptysis are less common. One such etiology includes mitral regurgitation. In patients with hemoptysis and unilateral consolidation, careful chest auscultation and cardiac assessment may assist in making an early diagnosis. [ABSTRACT FROM AUTHOR]

Copyright of Salud, Ciencia y Tecnología is the property of Fundacion Salud, Ciencia y Tecnologia 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.)

Human COPA mutations affecting retrograde Golgi-to-endoplasmic reticulum (ER) protein transport cause diffuse alveolar hemorrhage (DAH) and ER stress ("COPA syndrome"). Patients with SLE also can develop DAH. C57BL/6 (B6) mice with pristaneinduced lupus develop monocyte-dependent DAH indistinguishable from human DAH, whereas BALB/c mice are resistant. We examined Copa and ER stress in pristane-induced lupus. Copa expression, ER stress, vascular injury, and apoptosis were assessed in mice and COPA was quantified in blood from patients with SLE. Copa mRNA and protein expression were impaired in B6 mice with pristane-induced DAH, but not in pristane-treated BALB/c mice. An ER stress response (increased Hsp5a/BiP, Ddit3/CHOP, Eif2a, and spliced Xbp1) was seen in lungs from pristane-treated B6, but not BALB/c, mice. Resistance of BALB/c mice to DAH was overcome by treating them with low-dose thapsigargin plus pristane. CB6F1 mice did not develop DAH or ER stress, suggesting that susceptibility was recessive. Increased pulmonary expression of von Willebrand factor (Vwf), a marker of endothelial injury, and the chemokine Ccl2 in DAH suggested that pristane promotes lung microvascular injury and monocyte recruitment. Consistent with that possibility, lung endothelial cells and infiltrating bone marrow-derived cells from pristane-treated B6 mice expressed BiP and showed evidence of apoptosis (annexin-V and activated caspase-3 staining). COPA expression also was low in patients with SLE with lung involvement. Pristane-induced DAH may be initiated by endothelial injury, resulting in ER stress, apoptosis of lung endothelial cells, and recruitment of myeloid cells that propagate lung injury. The pathogenesis of DAH in SLE and COPA syndrome may overlap. [ABSTRACT FROM AUTHOR]

Glycoprotein IIb/IIIa inhibitors play a key role in the treatment of patients who have acute coronary syndromes and undergone percutaneous coronary intervention. However, its serious complication is diffused alveolar hemorrhage. A 73-year-old diabetic woman presented with chest pain and dynamic ST elevation in ECG and positive troponin. She had occlusion in two coronary arteries and underwent percutaneous coronary intervention. The eptifibatide was administered. After hours, she showed respiratory symptoms, as well as drop of blood pressure and hemoglobin. All differential diagnoses suggested for her clinical presentation were evaluated, and finally, on the sixth day diffuse alveolar hemorrhage was diagnosed. Although respiratory symptoms such as hemoptysis and dyspnea may occur as complications of pulmonary edema and/or pneumonia, assumed clinical suspicion for pulmonary hemorrhage leading to early detection of it. Moreover, there is no definitive guideline for decreased bleeding complications and treatment of alveolar hemorrhage caused by glycoprotein IIb/IIIa receptor inhibitors. [ABSTRACT FROM AUTHOR]

Copyright of Respiratory Case Reports is the property of LookUs Scientific 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.)

Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple organs. SLE can affect the lung, the pulmonary vasculature, and the pleura. A 38-year-old female with limb pain and ecchymosis who later developed pulmonary thromboembolism and alveolar hemorrhage is presented here. Clinical, imaging, laboratory, and histopathological evidence is presented. The patient met the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) criteria for SLE. Furthermore, the patient had a Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score of 35; thus, indicating severe disease. This case is an example of concomitant venous and arterial lung complications in an SLE patient. [ABSTRACT FROM AUTHOR]

Background and objectives: Bronchoalveolar lavage (BAL) is commonly performed to evaluate diffuse lung disease and occasionally to identify alveolar hemorrhage. However, the clinical impact of alveolar hemorrhage and its risk factors in patients with diffuse lung disease have not been clarified. Materials and Methods: We retrospectively analyzed the medical records of all patients who underwent BAL to evaluate diffuse lung disease from January 2017 to December 2020. Alveolar hemorrhage was defined as progressive hemorrhagic BAL fluid or the presence of ≥20% hemosiderin-laden macrophages in the BAL fluid. Logistic regression analysis was performed to assess the association between alveolar hemorrhage and other factors. Results: Sixty subjects were enrolled in this study. Alveolar hemorrhage was observed in 19 subjects (31.7%) with idiopathic interstitial pneumonia, acute respiratory distress syndrome, interstitial pneumonia with autoimmune features, drug-induced lung injury, eosinophilic pneumonia, adenocarcinoma, and systemic lupus erythematosus. The use of anticoagulants was a significant risk factor for alveolar hemorrhage (odds ratio 7.57, p = 0.049). Patients with alveolar hemorrhage required intubated mechanical ventilation more frequently (63.2% vs. 24.4%, p = 0.005) and had higher in-hospital mortality rates (26.3% vs. 4.9%, p = 0.028) than those without alveolar hemorrhage. Conclusions: Alveolar hemorrhage was observed in various etiologies. The use of anticoagulants was a significant risk factor for alveolar hemorrhage. Patients with alveolar hemorrhage showed more severe respiratory failure and had higher in-hospital mortality than those without alveolar hemorrhage. [ABSTRACT FROM AUTHOR]

Objective: To evaluate histomorphological effects of Soybean oil supplementation on lung tissue injury and alveolar hemorrhage induced by Bisphenol A (BPA). Study Design: Laboratory-based experimental study. Place and Duration of Study: Anatomy department, Army Medical College, Rawalpindi and National Institute of Health, Islamabad, from Nov 2015 to Nov 2016. Methodology: Forty (40) healthy BALB/c mice of 9-11 weeks of age, weighing between 30-37gm were housed in a controlled environment at National Institute of Health. Group 1 (10) was control group. Group 2 (10) was given a daily dose of 50 milligram/kilogram body weight of Bisphenol A and group 3 (10) was given a daily dose of 500 milligram of Soybean oil and group 4 (10) was concurrently given Bisphenol A and Soybean oil with daily doses of 50 milligram/kilogram body weight and 500 milligrams. After a period of 8 weeks, animals were dissected 24 hours after receiving the last dosage. Lung wet weight, animal weight and relative body tissue weight index (RTBWI) were calculated. Tissue processing & staining was done. Alveolar hemorrhage was histomorphologically and statistically analysed using SPSS-21. Results: On microscopic examination, alveolar hemorrhage (AH) was observed in 10 (100%) group 2 specimens with increase in RTBWI and whereas only 5 (50%) of group 4 specimens had alveolar hemorrhage with slight improvement in relative body tissue weight index (RTBWI). Conclusion: Bisphenol A (BPA) induced lung injury as evident by intraalveolar hemorrhage, blood vessel congestion and increased RTBWI ratio were ameliorated by concomitant administration of Soybean oil. [ABSTRACT FROM AUTHOR]

Copyright of Fukushima Medical Journal is the property of Medical Association of Fukushima 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.)

We described a case of alveolar hemorrhage (AH) after marijuana smoking using a water pipe made with plastic bottle (bong) before making a narrative review of the literature. A 19-year-old male was admitted for hemoptysis and dyspnea evolving since the previous day. He smoked marijuana ten times a day using bongs. Computed tomography scan of the chest (chest CT-scan) evidenced ground glass opacities involving upper lobes with crazy-paving pattern. Bronchoalveolar lavage (BAL) yielded fluid becoming progressively bloody suggestive of AH. Screening of drug metabolites ruled out the presence of cocaine degradation products. Treatment with prednisone was prescribed and oxygen requirements decreased rapidly. The patient accepted to stop bongs, but kept on smoking marijuana using joints. He was asymptomatic 2 months later; all ground glass opacities had vanished. Four cases described exactly the same circumstances as ours. All were young male patients containing ground glass opacities with diffuse or bilateral pattern in their chest CT-scan. The explanation suggested by the authors of these cases was the potential concomitant inhalation of acid anhydrides derived from use of heated plastic bottle. No acid anhydrides were experimentally evidenced after thermodesorption of heated polyethylene terephthalate (PET) (in which a majority of plastic bottles are made) we performed, but other compounds were. E-cigarette, or vaping, product use-associated lung injuries cases share some chest CT-scan patterns with those of AH following bong use and we tried to draw a parallel between these two latter before discussing a physiopathological hypothesis. [ABSTRACT FROM AUTHOR]

Mendelian autoinflammatory diseases characterized by constitutive activation of the type I interferon pathway, the so-called type I interferonopathies, constitute a rapidly expanding group of inborn errors of immunity. Among the type I interferonopathies, STING-associated vasculopathy with onset in infancy (SAVI) and COPA syndrome were described in the last 6 years, both manifesting a major inflammatory lung component associated with significant morbidity and increased mortality. There is striking clinical and histopathological overlap between SAVI and COPA syndrome, although distinct features are also present. Of note, there is a remarkably high frequency of clinical non-penetrance among individuals harboring pathogenic COPA mutations. SAVI is caused by, principally heterozygous, gain-of-function mutations in STING1 (previously referred to as TMEM173) encoding STING, a key adaptor of the interferon signaling pathway induced by DNA. COPA syndrome results from heterozygous dominant-negative mutations in the coatomer protein subunit alpha, forming part of a complex involved in intracellular cargo protein transport between the Golgi and the endoplasmic reticulum (ER). Of importance, a role for COPA in regulating the trafficking of STING, an ER-resident protein which translocates to the Golgi during the process of its activation, was recently defined, thereby possibly explaining some aspects of the phenotypic overlap between SAVI and COPA syndrome. Here, we review the expanding phenotype of these diseases, highlighting common as well as specific features, and recent advances in our understanding of STING biology that have informed therapeutic decision-making in both conditions. Beyond these rare Mendelian disorders, DNA sensing through STING is likely relevant to the pathology of several diseases associated with lung inflammation, including systemic lupus erythematosus, dermatomyositis, environmental toxin exposure, and viral infection. [ABSTRACT FROM AUTHOR]

Advances in the diagnosis and treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis have led to continued improvement in survival and prognosis over the course of the last 4 decades. Nevertheless, the most acute and severe disease manifestations, including severe kidney disease and alveolar hemorrhage, continue to be associated with increased early mortality from disease activity or treatment complications as well as risk for the development of end-stage kidney disease (ESKD), which in turn directly affects the overall prognosis of ANCA-associated vasculitis. Plasma exchange (PLEX) has long been proposed and used for these most severe disease manifestations under the assumption that its effects are swift and supported by our understanding of the pathogenic role of ANCA. Yet convincing evidence of a beneficial effect of PLEX in ANCA-associated vasculitis has been lacking, as early studies and small trials have generated conflicting results. The controversy regarding PLEX has been accentuated recently as the largest randomized controlled trial ever conducted in ANCA-associated vasculitis, the Plasma Exchange and Glucocorticoids in Severe ANCA-associated Vasculitis trial, which was specifically designed to evaluate the efficacy of PLEX in patients with severe renal disease or alveolar hemorrhage, failed to show a difference in the combined primary outcome measure of death or ESKD in patients who received PLEX versus those who did not. In light of these disappointing results, we herein review the currently available data on PLEX for ANCA-associated vasculitis and explain why we believe that these data no longer support the use of PLEX in ANCA-associated vasculitis. [ABSTRACT FROM AUTHOR]

Background: Extracorporeal membrane oxygenation (ECMO) is associated with complications that are separate from the underlying diagnoses that require its use. One of the foremost complications of ECMO is a high incidence of bleeding, including alveolar hemorrhage (AH), which is believed to be due to both prophylactic anticoagulation and critical illness-induced systemic coagulopathy. However, akin to systemic inflammatory response syndrome after cardiopulmonary bypass, ECMO causes widespread systemic inflammation and acute lung injury, which likely further predisposes patients to AH. The burden of clinically significant AH among patients on ECMO for advanced lung disease remains unknown. Patients and methods: Charts of patients with advanced lung disease who required ECMO at a single institution were reviewed. The clinical course and variables of patients who developed AH and those who did not were compared. Results: This report describes five patients who developed AH after initiation of venovenous ECMO for refractory hypoxemia. Clinical and laboratory variables did not predict the development or the prognosis of AH. Two of these patients with refractory hypoxemia and AH were treated with pulse-dose corticosteroids, with a dramatic response in one case. Conclusion: The acute decompensation of the patients and response to corticosteroids suggest AH was mediated by a systemic inflammatory process, as opposed to coagulopathy alone. Judicious use of steroids may be considered among select patients who develop AH without symptoms of systemic coagulopathy after initiation of ECMO. [ABSTRACT FROM AUTHOR]

A 36-year-old female presented with two episodes of hemoptysis induced by exertion and acute dyspnea. She was diagnosed as a case of hypertrophic obstructive cardiomyopathy (HOCM) with systolic anterior motion (SAM)-induced severe mitral regurgitation (MR). She underwent extended septal myectomy with mitral valve replacement under cardiopulmonary bypass and recovered successfully. This is a unique and rare combination of HOCM with hemoptysis. [ABSTRACT FROM AUTHOR]

A 31-year non-smoker man, working in plastic making industry for 12 years presented with cough and streaking hemoptysis for 2 days. Computed tomography (CT) of chest showed patchy ground glass opacities with interlobular septal thickening in bilateral lung parenchyma. Fiber optic bronchoscopy (FOB) was done. Sequential lavage was taken which showed progressively increasing hemorrhagic fluid. His diffusion capacity for carbon monoxide (DLCO) was 38.08 mL/mmHg/Mi (126%) predicted on day 2 of admission, 32.36 ml/mmHg/Mi (106%) predicted on discharge and 39.63 mL/mmHg/Mi (130%) predicted on going back to work. He was diagnosed with plastic fume exposure related pulmonary alveolar hemorrhage. [ABSTRACT FROM AUTHOR]

Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-ranging pathological mechanism in PAH could elucidate the clinical state of severe CHD-PAH. Using electronic medical records, we retrospectively analyzed six infants with severe CHD-PAH who had treatment-resistant PH. All patients were born with congenital malformation syndrome. After starting on a pulmonary vasodilator, five of the six patients developed complications including pulmonary edema and interstitial lung disease (ILD), and four patients had alveolar hemorrhage. After steroid therapy, the clinical condition improved in four patients, but two patients died. The autopsy findings in one of the deceased patients indicated the presence of recurrent alveolar hemorrhage, pulmonary venous hypertension, ILD, and PAH. Based on the clinical course of these CHD-PAH in patients and the literature, CHD-PAH can occur with pulmonary vascular obstructive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH), ILD, and/or alveolar hemorrhage. The severity of CHD-PAH may depend on a genetic disorder, respiratory infection, and upper airway stenosis. Additionally, pulmonary vasodilators may be involved in the development of PVOD/PCH and ILD. When patients with CHD-PAH show unexpected deterioration, clinicians should consider complications associated with PVOD/PCH and/or pulmonary disease. In addition, the choice of upfront combination therapy for pediatric patients with CHD-PAH should be selected carefully. [ABSTRACT FROM AUTHOR]

Vasculitides associated with anti-neutrophil cytoplasmic antibodies are heterogeneous, systemic, low prevalence and high morbidity and mortality entities. They include granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. In Latin America, there are few descriptive registries of these patients. The objective of the study was to describe the demographic and clinical characteristics and in-hospital morbidity and mortality of patients with vasculitis associated with anti-neutrophil cytoplasmic antibodies in a university hospital in Colombia. This was a cross-sectional descriptive study. We performed computer searches with terms related to patients with anti-neutrophil cytoplasmic antibody-associated vasculitis, between 2005 and 2017 who met the American College of Rheumatology classification criteria for vasculitis associated with anti-neutrophil cytoplasmic antibodies, and their clinical and laboratory characteristics. One hundred and six patients with anti-neutrophil cytoplasmic antibody-associated vasculitis were included in the study. The average age was 55 years, and 57.5% were women. In 68.8% of the cases, the diagnosis was made during hospitalization, with an average hospital stay of 16.6 days (± 12.22). The distribution by type of vasculitis was: granulomatosis with polyangiitis 52%, microscopic polyangiitis 45.2% and eosinophilic granulomatosis with polyangiitis 1.8%. Alveolar hemorrhage occurred in 35% of patients; 20.7% had variable renal involvement, of which 53.8% progressed to advanced kidney disease. Treatment included glucocorticoids 91.5%, cyclophosphamide 62.2%, plasmapheresis 14.1%, and 41.5% required renal replacement therapy. In-hospital mortality was 16.5%, Sepsis was the most common cause of death. We present clinical information on a group of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis; renal involvement was the the most common type of affectation. Both the clinical and serological characteristics of our registry were similar to those described in other Latin American and European cohorts, and a lower in-hospital mortality rate was evidenced. [ABSTRACT FROM AUTHOR]

Pulmonary Alveolar Proteinosis (PAP) is a rare lung disease characterized by accumulation of a lipoproteinous material in the alveoli and distal airways. Antibodies against granulocyte macrophage colony stimulant factor (GM-CSF) are thought to be responsible for its pathogenesis. Antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of vasculitis affecting small and medium vessels and often characterized by lung and kidney involvement. Here, we present a patient with PAP diagnosis who has been in remission for many years, developing AAV and associated pulmonary alveolar hemorrhage. [ABSTRACT FROM AUTHOR]

Purpose of Review: Diffuse alveolar hemorrhage (DAH) is a rare but devastating manifestation of antiphospholipid syndrome (APS) patients with or without other systemic autoimmune diseases. Data regarding diagnosis and treatment are limited to case series. We review diagnostic and therapeutic strategies employed in APS patients with DAH and discuss our experience in managing these complex patients. Recent Findings: Pulmonary capillaritis likely contributes to the pathogenesis, however is only observed in half of the biopsies. Corticosteroids induce remission in the majority of patients, however almost half recur and require a steroid-sparing immunosuppressive to maintain remission. Cyclophosphamide- or rituximab-based regimens achieve the highest remission rates (50%); other strategies include intravenous immunoglobulin, plasmapheresis, mycophenolate mofetil, and/or azathioprine. Summary: Given the rarity of DAH in APS, treatment is guided by interdisciplinary experience. Why certain patients achieve full remission with corticosteroids while others require immunosuppressive agents is unknown; future research should focus on the pathophysiology and optimal management. [ABSTRACT FROM AUTHOR]

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Copyright of Zeitschrift für Herz-, Thorax- und Gefaesschirurgie 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.)

El síndrome pulmón-riñón rara vez ha sido reportado como cuadro clínico de presentación de vasculitis causada por el consumo de cocaína contaminada con levamisol. Se reporta el caso de un paciente con este cuadro clínico y se señalan las dificultades que se presentaron durante el abordaje diagnóstico y terapéutico. Pulmonary-renal syndrome has rarely been reported as the clinical presentation of vasculitis caused by the consumption of cocaine adulterated with levamisole. We report the case of a patient in whom we detected the clinical manifestations and indicate the difficulties that arose in relation to the diagnostic and therapeutic approach. [ABSTRACT FROM AUTHOR]

Strongyloides stercoralis is an intestinal nematode that occurs sporadically in temperate areas like Korea. People who are in the immunosuppressed state, over the age of 65 or under the corticosteroid therapy are at risk for developing Strongyloides hyperinfection syndrome. Acute respiratory distress syndrome (ARDS) with alveolar hemorrhage is a rare presentation of Strongyloides hyperinfection. A 78-year-old man had been irregularly injected corticosteroid on his knees, but did not have any immunosuppressive disease. He was initially diagnosed with ARDS and septic shock. Bronchoalveolar lavage (BAL) fluid was bloody and its cytology revealed helminthic larvae identified as S. stercoralis. Results of Cytomegalovirus polymerase chain reaction (PCR), Pneumocystis jirovecii PCR, and Aspergillus antigen testing of the BAL fluid were positive. The clinical progress quickly deteriorated with multiple organ failure, shock and arrhythmia, so he finally died. This is a rare case of ARDS in an older patient without any known immunosuppressive conditions, with alveolar hemorrhage and S. stercoralis being found via BAL. [ABSTRACT FROM AUTHOR]

Copyright of Der Pneumologe 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.)

Copyright of Rassegna di Patologia dell'Apparato Respiratorio is the property of AIPO - Associazione Italiana Pneumologi Ospedalieri 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.)

We herein describe the case of a 67-year-old woman with advanced lung adenocarcinoma who developed interstitial lung disease (ILD) with alveolar hemorrhage induced by pembrolizumab. She received four courses of pembrolizumab therapy and achieved a partial response. She had no respiratory symptoms; however, chest radiography and computed tomography (CT) revealed ground-glass opacities (GGOs) and crazy-paving pattern. Based on findings of bloody bronchoalveolar lavage fluid and transbronchial lung biopsy samples, pembrolizumab-induced ILD with alveolar hemorrhage was diagnosed. Corticosteroid therapy rapidly improved alveolar hemorrhage and regressed GGOs on CT scan. This is the first report on ILD with alveolar hemorrhage induced by pembrolizumab. [ABSTRACT FROM AUTHOR]

Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Serological testing for anti-GBM antibodies may facilitate rapid diagnosis, though renal biopsy is often required to confirm the presence of necrotizing or crescentic GN and linear deposition of autoantibody on the glomerular basement membrane. Alveolar hemorrhage may be evident clinically, or detected on imaging, pulmonary function testing, or bronchoscopy. Prompt treatment with plasmapheresis, cyclophosphamide, and steroids is usually indicated to remove pathogenic autoantibodies, to prevent their ongoing production, and to ameliorate end-organ inflammation. Alveolar hemorrhage is usually responsive to this treatment, and long-term respiratory sequelae are uncommon. Renal prognosis is more variable, though with aggressive treatment, independent renal function is maintained at 1 year in more than 80% of patients not requiring renal replacement therapy at presentation. Relapse in uncommon in anti-GBM disease, unless there is a concomitant antineutrophil cytoplasm antibody (present in 30-40%), in which case maintenance immunosuppression is recommended. [ABSTRACT FROM AUTHOR]

Nail guns are cheap and easy-to-access devices that are common in the industry and personal use and are widely used by untrained people. Life-threatening injuries may occur after a self-accident due to the use of these devices. We report the case of a 47-year-old man who had hemoptysis due to a lung parenchymal injury after nail gun use. Computed tomography revealed focal alveolar hemorrhage. Hemodynamically stable, the patient underwent surgery. Nail guns penetrating the chest can cause life-threatening situations. In the emergency department, rapid diagnosis and treatment is vital in preventing fatal outcome. [ABSTRACT FROM AUTHOR]

Introduction: DAH is a clinical condition that can lead to dyspnea, hemoptysis, anemia and alveolar consolidations and cause acute respiratory failure. DAH may occur due to immune and non-immune causes, it is rare to develop as a result of IVIG treatment. Herein, a case of DAH diagnosed as a result of IVIG treatment is presented. Case: A 46-year-old male patient admitted to the emergency room with complaints of shortness of breath and cough was admitted to the intensive care unit after a positive covid test. The patient had a history of ischemic stroke and was taking acetyl salicylic acid and clopidogrel. On physical examination SpO2 : 80% and decreased breath sounds in bilateral lungs. Laboratory results showed WBC: 27 K/uL, HGB: 9 g/dL PLT: 107 K/uL, procalcitonin: 10 ug/L CRP: 175 mg/L. Thorax CT of the patient showed ground-glass density and fibrotic changes in the lower lobes of both lungs. The patient was started on piperacillin tazobactam 4x2.25 gr, moxifloxacin 1x400 mg, prednisolone 1x80 mg, enoxaparin IU/0.4 mL 1x1, pantoprazole 1x40 mg. Patient with HGB: 8 g/dL, PLT: 1 K/uL with extensive petechiae on the body by performing a peripheral blood smear hematology department was consulted and low platelets were attributed to sepsis. After 2 days of 80g IVIG, the patient’s PLT value started to increase, but hemoptysis occurred. Continuous positive airway pressure was applied to the patient who had diffuse consolidation in both lungs compatible with DAH on thorax CT. The patient who did not have hemoptysis in the follow-up, whose consolidations decreased in PAAC and whose antibiotic treatment was completed was discharged with the recommendation of outpatient clinic control. Discussion: IVIG is a plasma product widely used in the treatment of many autoimmune and systemic inflammatory diseases. However, serious side effects such as acute renal failure, myocardial infarction, stroke, deep vein thrombosis, pulmonary embolism, anaphylaxis and aseptic meningitis may occur. DAH, after IVIG has also been reported as a very rare complication. [ABSTRACT FROM AUTHOR]

Influenza A virus infection is a common respiratory tract infection. Alveolar hemorrhage has been reported in patients with influenza pneumonia and in mice infected with influenza A. In this study, we investigated the effect of two anticoagulants on alveolar hemorrhage after influenza A virus (IAV) infection of wild-type mice. Wild-type mice were anticoagulated with either warfarin or the direct thrombin inhibitor dabigatran etexilate and then infected with a mouse-adapted influenza virus (A/Puerto Rico/8/34 H1N1). Alveolar hemorrhage was assessed by measuring hemoglobin levels in the bronchoalveolar lavage fluid (BALF). We also measured vascular permeability and viral genomes in the lung, as well as white blood cells, inflammatory mediators, and protein in BALF. Survival and body weight were monitored for 14 days after influenza A infection. In infected mice receiving either warfarin or dabigatran etexilate we observed decreased activation of coagulation in the BALF and increased alveolar hemorrhage. Warfarin but not dabigatran etexilate increased vascular permeability and mortality of influenza A-infected mice. Anticoagulation did not affect levels of influenza A genomes, white blood cells, inflammatory mediators, or protein in the BALF. Our study indicates that systemic anticoagulation increases alveolar hemorrhage in influenza A-infected mice. [ABSTRACT FROM AUTHOR]

The main clinical manifestations of wild-type transthyretin (TTR)-related amyloidosis are progressive heart failure and neuropathy. There have been some reports on cerebral hemorrhage due to cerebral amyloid angiopathy in patients with TTR-related amyloidosis, but little is known about the vascular involvement in other organs. A 77-year-old woman expe rienced heart failure and was admitted for deteriorating heart failure status. Echocardiography showed diffuse hypokinesis of the left ventricle with biventricular wall thickness. On the 12th hospital day, the blood oxygen saturation level suddenly dropped and, despite oxygen supplementation and intensive care, the patientdied. Anautopsy revealed systemic deposition of amyloids which were immunolabeled by an anti-TTR antibody. Furthermore, gene-sequencing analysis showed no evidence of TTR gene mutations. The patient was diagnosed postmortem with wild-type TTR-related amyloidosis. Pathological findings revealed alveolar hemorrhage of the lung. Massive amyloid deposits were present in the vessels, and collapsed internal elastic laminae with lymphocyte infiltration were observed at the site of amyloid deposits in the bronchial artery, suggesting that deposits with inflammation might cause the collapse of the bronchial artery and lead to hemorrhage. In amyloidosis patients who suffer heart failure, there is the potential for vascular collapse caused by the accumulation of amyloid deposits with inflammation. [ABSTRACT FROM AUTHOR]

Copyright of Archives of the Turkish Society of Cardiology / Türk Kardiyoloji Derneği Arşivi is the property of KARE Publishing 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.)

Over the last decades, post-infectious glomerulonephritis underwent major changes in its epidemiology, pathophysiology, and outcomes. We are reporting a case of IgA-dominant post-infectious glomerulonephritis (IgA-PIGN) presenting as a fatal pulmonary-renal syndrome. An 86-year-old Filipino man presented with worsening dyspnea, hemoptysis, and decreased urine output over 2 weeks. Past medical history is significant for hypertension, chronic kidney disease stage III, and pneumonia 3 weeks prior treated with intravenous cefazolin for methicillinsensitive Staphylococcus aureus bacteremia. Physical examination was remarkable for heart rate of 109/min and respiratory rate of 25/min saturating 99% on 3 liters via nasal cannula. There were bibasilar rales in the lungs and bilateral ankle edema. A chest radiograph showed bibasilar opacifications. Blood work was significant for hemoglobin of 8.3 g/dL and creatinine of 9.2 mg/ dL (baseline of 1.67). TTE showed EF 55%. Urinalysis revealed large blood and red blood cell casts. Kidney ultrasound showed bilateral echogenicity compatible with renal disease. Pulse methylprednisolone therapy and hemodialysis were initiated with patient's condition precluding kidney biopsy. Serology workup for rapidly progressive glomerulonephritis was negative. On day 7, the patient required mechanical ventilation; bronchoscopy showed alveolar hemorrhage and plasmapheresis was initiated. Renal biopsy revealed IgA-PIGN with endocapillary and focal extracapillary proliferative and exudative features. IgA-PIGN occurs in diabetic elderly (mean age of 60 years), 0-16 weeks after an infection mainly by Staphylococcus. However, this nondiabetic patient had normal complement IgA-PIGN with fatal pulmonary-renal syndrome. Understanding the pathogenesis and identifying the nephrotoxic bacteria species and the aberrant IgA molecule will open new insights toward prevention and treatment. [ABSTRACT FROM AUTHOR]

Hemorrhagic complications of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis are life-threatening conditions. Of these, alveolar hemorrhage is the most common and well-described hemorrhagic complication and is a prominent component of the pulmonary-renal syndrome. The clinical presentation of alveolar hemorrhage is highly variable ranging from mild to serious disease that may require respiratory support. Less attention has been directed at hemorrhagic complications that have been reported in other organs and which may be as severe or more severe with regard to morbidity and mortality. For example, among the most serious non-pulmonary complications are those related to cerebral and gastrointestinal hemorrhage. Cerebral hemorrhage is the clearly the most critical issue with a persistent high mortality rate. The prognosis of gastrointestinal hemorrhage, once an ominous complication has improved with medical and surgical intervention. Not to be dismissed are the consequences of hemorrhagic issues related to skin, soft tissue or muscle involvement. Muscle hematomas, while rare, also accompanied by significant morbidity. [ABSTRACT FROM AUTHOR]

Background. Pulmonary involvement is one of the hallmark lesions of anti-neutrophil cytoplasmic autoantibody (ANCA)- associated vasculitis (AAV) as well as rapidly progressive glomerulonephritis (RPGN). However, the pulmonary involvement of AAV patients seems to differ between Europe and Japan, as does the ANCA serotype. Methods. This retrospective and prospective multicenter cohort study collected the clinical data of the features and outcomes of 1772 RPGN patients treated from 1989 to 2007 in Japan. Based on this nationwide RPGN survey, we analyzed the cases of 1147 AAV patients. Results. We found that 52.3% of the AAV patients had pulmonary involvements: 15.4% of the AAV patients had alveolar hemorrhage (AH), 26.2% had interstitial lung disease (ILD), 2.8% had bronchial asthma, 2.4% had pulmonary granuloma and 12.8% had a chest X-ray abnormality without AH, ILD or pulmonary granuloma. Patient survival was significantly different among the following six groups: the 5-year survival rate was 41.5% in the patients with AH, 50.2% in those with ILD, 67.9% in those with bronchial asthma, 62.5% in those with pulmonary granuloma, 55.8% in those with chest X-ray abnormality and 73.3% in those without pulmonary involvement. AH was one of the predictors of 1- and 5-year mortality for patient survival in AAV, and ILD was added as one of the predictors of 5-year mortality. Conclusion. In these AAV patients, not only AH but also ILD was frequently observed. AH was associated with the prognosis, but ILD was associated with the long-term prognosis of AAV. [ABSTRACT FROM AUTHOR]

We report a case of a 58-year-old male without any comorbidities who was thrombolysed with streptokinase for acute anterior wall myocardial infarction and developed massive hemoptysis with dyspnea and imaging features showing bilateral alveolar infiltrates. He was diagnosed with pulmonary alveolar hemorrhage and treated conservatively. His condition improved, and follow-up imaging showed resolution. Alveolar hemorrhage is a rare and lifethreatening complication of thrombolytic therapy. [ABSTRACT FROM AUTHOR]

Illicit stimulants, such as cocaine, amphetamine, and their derivatives (e.g., 'ecstasy'), continue to exact heavy toll on health care in both developed and developing countries. The US Department of Health and Human Service reported over one million illicit drug-related emergency department visits in 2010, which was higher than any of the six previous years. Both inhaled and intravenous forms of these substances of abuse can result in a variety of acute and chronic injuries to practically every part of the respiratory tract, leading potentially to permanent morbidities as well as fatal consequences-including but not limited to nasal septum perforation, pulmonary hypertension, pneumothorax, pneumomediastinum, interstitial lung disease, alveolar hemorrhage, reactive airway disease, pulmonary edema, pulmonary granulomatosis, infections, foreign body aspiration, infections, bronchoconstriction, and thermal injuries. Stimulants are all rapidly absorbed substances that can also significantly alter the patient's systemic acid-base balance and central nervous system, thereby leading to further respiratory compromise. Mounting evidence in the past decade has demonstrated that adulterants coinhaled with these substances (e.g., levamisole) and the metabolites of these substances (e.g., cocaethylene) are associated with specific forms of systemic and respiratory complications as well. Recent studies have also demonstrated the effects of stimulants on autoimmune-mediated injuries of the respiratory tract, such as cocaine-induced midline destructive lesions. A persistent challenge to studies involving stimulant-associated respiratory toxidromes is the high prevalence of concomitant usage of various substances by drug abusers, including tobacco smoking. Now more than ever, health care providers must be familiar with the multitude of respiratory toxidromes as well as the diverse pathophysiology related to commonly abused stimulants to provide timely diagnosis and effective treatment. [ABSTRACT FROM AUTHOR]

Copyright of Acta Medica Colombiana is the property of Acta Medica Colombiana 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.)

Copyright of Reumatología Clínica is the property of Elsevier B.V. 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.)