Diagnosis Of Diffuse Alveolar Hemorrhage Research Articles

Juvenile idiopathic arthritis presenting as diffuse alveolar hemorrhage at onset: A case series.

The etiology of diffuse alveolar hemorrhage (DAH) in childhood is often unknown, and it may be an early manifestation of rheumatic disease. Juvenile idiopathic arthritis (JIA) is one of the most common rheumatic diseases in children, but DAH as an onset manifestation of JIA is rare. This study summarizes the clinical characteristics of patients with JIA presenting as DAH. We retrospectively analyzed the age of onset, clinical manifestations, imaging features, treatments, and prognosis of five cases of JIA presenting as DAH. Themedian age at DAH onset was 6 months (range, 2 months-3 years). Pallor was the most common manifestation of onset (5/5). Other symptoms included cough (2/5), tachypnea (2/5), hemoptysis (1/5), cyanosis (1/5), and fatigue (1/5). Imaging showed ground-glass opacity (GGO) (5/5), subpleural or intrapulmonary honeycombing (4/5), consolidation (3/5), interlobular septal thickening (2/5), and nodules (1/5). Anticitrullinated protein antibodies (ACPA) and rheumatoid factor (RF) were positive in five children (5/5), and antinuclear antibody (ANA) was positive in four children (4/5). ANA in three children and ACPA/RF in one child were positive before the onset of joint symptoms. The median age at the onset of joint symptoms was 3 years and 9 months (2 years and 6 months-8 years). Joint symptoms were mainly characterized by joint swelling, pain, and difficulty walking, and the most commonly affected joints were the knees, ankles, and wrists. After the diagnosis of DAH, the five patients were treated with glucocorticoids. Alveolar hemorrhage was effectively controlled in three cases, but the other two patients still had anemia and poor improvements in chest imaging. After joint symptoms, the patients were treated with glucocorticoids combined with diclofenac, disease-modifying antirheumatic drugs, and biological agents. Alveolar hemorrhage was in remission, and joint symptoms were relieved in the five cases. DAH can be the first clinical manifestation of JIA, and joint involvement occurs 1-5 years later. Children with DAH who are positive for RF, ACPA, and/or ANA and have GGO accompanied by honeycombing on imaging should be concerned about their joint involvement in future.

Diffuse alveolar hemorrhage secondary to sarcoidosis.

Diffuse alveolar hemorrhage (DAH) is a pulmonary condition that can be caused by autoimmune disorders such as lupus, small vessel vasculitis, and antiphospholipid syndrome. Sarcoidosis as a cause of DAH has been reported; however, the literature remains limited. We performed a chart review for patients with a diagnosis of both sarcoidosis and DAH. Seven patients met inclusion criteria. Mean (range) patient age was 54years (39-72), and 3 patients had a history of tobacco use. Diagnosis of DAH and sarcoidosis were concurrent for 3 patients. Corticosteroids were used for treatment of DAH in all patients; 2 (including 1 with refractory DAH) were successfully treated with rituximab. We believe sarcoidosis-associated DAH is more common than previously reported. It is essential to consider sarcoidosis in the differential diagnosis of immune-mediated DAH. Key Points • Sarcoidosis can cause diffuse alveolar hemorrhage (DAH); more extensive studies are needed to estimate this condition's prevalence. • BMI of 25 or higher appears to be a risk factor for the development of sarcoidosis-associated DAH.

Rheumatic diseases associated with alveolar hemorrhage: analysis of the national inpatient sample.

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. 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. 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 61.8years and a mean LOS of 10.6days. 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). 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.

A RARE MANIFESTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS (SLE): DIFFUSE ALVEOLAR HEMORRHAGE

A RARE MANIFESTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS (SLE): DIFFUSE ALVEOLAR HEMORRHAGE

Metagenomic next-generation sequencing in the diagnosis of leptospirosis presenting as severe diffuse alveolar hemorrhage: a case report and literature review

BackgroundLeptospirosis is a common infectious disease in tropical and semitropical regions, and it is typically neglected. Leptospirosis-associated acute diffuse alveolar hemorrhage is one of its fatal complications. The use of bronchoalveolar lavage fluid (BALF) metagenomic next-generation sequencing in the diagnosis of Leptospira interrogans infection has rarely been reported.Case presentationWe present the case of a 62-year-old female who was transferred to our hospital with dyspnea, and severe hemoptysis and was supported by a tracheal intubation ventilator. Bronchoalveolar lavage fluid (BALF) metagenomic next-generation sequencing (mNGS) reported Leptospira interrogans. A diagnosis of diffuse alveolar hemorrhage caused by leptospirosis was made. After immediately receiving antibiotics and hormone therapy, the patient achieved a complete recovery upon discharge.ConclusionLeptospirosis presenting as severe diffuse alveolar hemorrhage is rare but should be considered in the differential diagnosis. mNGS can help identify pathogens and treat them early, which can improve prognosis.

DIFFUSE ALVEOLAR HEMORRHAGE RESULTING FROM RIVAROXABAN USE: A RARE COMPLICATION IN A PATIENT WITHOUT PREDISPOSING CONDITIONS

TOPIC: Diffuse Lung Disease TYPE: Fellow Case Reports INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a rare complication of direct oral anticoagulant (DOAC) use with few reported cases in patients with predisposing conditions or underlying lung disease. DAH results from disruption of the alveolar-capillary interface. Infectious, autoimmune, and drug mediated conditions may lead to DAH. Rivaroxaban is a DOAC used to treat venous thromboembolism (VTE) and non-valvular atrial fibrillation (NVAF). We present a case of a patient who developed DAH one month after starting rivaroxaban. CASE PRESENTATION: A 43-year-old female with a past medical history of hyperlipidemia and tobacco use disorder presented with frank hemoptysis and shortness of breath. She was diagnosed with lower extremity deep vein thrombosis (DVT) and was started on rivaroxaban. Vitals were stable without hypoxia. Hemoglobin dropped from 14 to 7 g/dL (normal 12.3-15.3 g/dL) requiring 2 units of blood. Prothrombin Complex Concentrate was given. Computed tomography angiogram of the chest showed bilateral lung fields with patchy ground-glass opacities with peripheral sparing, without significant intra-lobular septal thickening and no pulmonary embolus. Infectious work up was negative. Rheumatological workup with anti-glomerular basement antibodies, anti-double stranded DNA antibodies, lupus anticoagulant, antinuclear antibody, rheumatoid factor, anti-neutrophilic cytoplasmic antibody, proteinase-3 antibody, myeloperoxidase antibody, C-reactive protein and erythrocyte sedimentation rate was negative. Bronchoscopy confirmed the diagnosis of DAH. Hemoptysis resolved after discontinuing rivaroxaban, repeat lower extremity dopplers were negative for DVT. She was discharged home without anticoagulation. DISCUSSION: DAH occurs from direct injury or inflammation of the pulmonary arterioles, venules, or alveolar septal capillaries causing bleeding into the lung. It presents with non specific symptoms including cough, chest pain, hemoptysis, and dyspnea. It is life threatening with fast clinical deterioration leading to respiratory failure. The underlying vascular injury of DAH is based on histological appearance and is divided into pulmonary capillaritis, bland pulmonary hemorrhage, and diffuse alveolar damage. The diagnosis is usually made clinically and supported with imaging and bronchoscopic findings. There are few cases of rivaroxaban induced DAH in the literature. The majority of the cases report patients with an underlying medical conditions such as autoimmune disease, lung cancer, interstitial lung disease and pulmonary hypertension, predisposing them to DAH. Our patient had no predisposing factors that put her at risk of developing DAH. CONCLUSIONS: Oral anticoagulants are a under-recognized cause of DAH with limited literature on the incidence of DAH with the use of these medications. This case illustrates that patients with no underlying risk factors can develop DAH with the use of rivaroxaban. REFERENCE #1: Collard HR, Schwarz MI. Diffuse alveolar hemorrhage. Clinics in Chest Medicine 2004; 25(3): 583-92. REFERENCE #2: Park MS. Diffuse alveolar hemorrhage. Tuberc Respir Dis (Seoul) 2013; 74(4): 151-62. REFERENCE #3: Chen A, Stecker E, Warden BA. Direct Oral Anticoagulant Use: A Practical Guide to Common Clinical Challenges. Journal of the American Heart Association 2020; 9(13): e017559 DISCLOSURES: No relevant relationships by Ahmad Alhajhusain, source=Web Response No relevant relationships by Stephanie Baltaji, source=Web Response No relevant relationships by Helene Rovnan, source=Web Response

TAKE A CHILL PILL: CRYOBIOSPY USED TO DIAGNOSE ISOLATED PULMONARY CAPILLARITIS CAUSING DIFFUSE ALVEOLAR HEMORRHAGE

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Diffuse alveolar hemorrhage(DAH) is associated with three histopathologic patterns: pulmonary capillaritis (PC), bland pulmonary hemorrhage, and diffuse alveolar damage. PC is most commonly caused by systemic autoimmune processes and in the absence of evidence of systemic disease is known as isolated pulmonary capillaritis (IPC), which is limited to the lung. Transbronchial cryobiopsy is a bronchoscopic alternative to surgical lung and transbronchial biopsy that uses cryoforceps to quickly freeze at -196ºC to preserve it for immunohistochemical analysis. We describe a case of cryobiopsy used for histopathological diagnosis of IPC in a case of DAH. CASE PRESENTATION: A 70 year-old male presented to the ED with severe hemoptysis. He was intubated for acute hypoxic respiratory failure. CT Chest showed bilateral ground-glass and airspace opacities. BAL showed progressively hemorrhagic effluent on serial aliquots on 2 occasions, confirming diagnosis of DAH. He received high-dose IV steroids. Autoimmune work-up, including antinuclear antibody(ANA), ANCA antibody, myeloperoxidase antibody, proteinase-3 antibody, anti-double stranded DNA, anti-cyclic citrullinated peptide, anticardiolipin antibody, C3, C4, anti-ground basement membrane(GBM) were negative twice. Therefore, tissue diagnosis was pursued. Transbronchial cryobiopsy was performed. Biopsy revealed mild expansion of the alveolar walls, scattered foci of fibrin present within the airspaces, collections of hemosiderin-laden macrophages, focal areas of increased neutrophils within the capillary network of alveolar walls with reactive endothelial changes, and no evidence of large vessel vasculitis. In the setting of negative autoimmune workup, this indicated isolated pulmonary capillaritis. With mycophenolate mofetil and prolonged steroid taper, he recovered with no recurrence of hemoptysis on 6 month follow-up. DISCUSSION: IPC is a rare entity causing DAH with favorable prognosis when treated. Thus, pursuing tissue diagnosis in DAH with negative autoimmune workup has important treatment implications. Cryobiopsy has a diagnostic yield of 75-91%-superior to that of transbronchial lung biopsy, while surgical lung biopsy is a major invasive procedure with mortality rate of 1-5%. While the use of cryobiopsy remains limited to centers of expertise, with complications of pneumothorax and hemorrhage reported, it is a promising and cost-effective approach which can be considered to establish a diagnosis in DAH of unclear etiology. CONCLUSIONS: Cryobiopsy should be considered to establish a diagnosis in diffuse alveolar hemorrhage of unclear etiology. Reference #1: Thompson, Gwen, Mary Klecka, Anja C. Roden, Ulrich Specks, and Rodrigo Cartin-Ceba. "Biopsy-proven Pulmonary Capillaritis: A Retrospective Study of Aetiologies including an In-depth Look at Isolated Pulmonary Capillaritis.” Respirology 21.4 (2016): 734-38 DISCLOSURES: No relevant relationships by Raed Alalawi, source=Web Response No relevant relationships by Abdul Khan, source=Web Response No relevant relationships by Nehan Sher, source=Web Response No relevant relationships by Daniel Yu, source=Web Response

Dengue Infection Induced Diffuse Alveolar Hemorrhage

Background: Dengue fever is a commonly encountered arthropod-borne viral disease. The spectrum of the disease varies widely, and at times it may present with unusual complications. One such dreaded but rare complication is diffuse alveolar hemorrhage (DAH). Case Report: A 42 years old lady having no co-morbidities presented with dengue infection. The presentation included fever, gum bleed, and asthenia. The examination showed low oxygen saturation and clear chest. The discordance between clinical findings and the oxygen saturation led to a cascade of investigations, culminating in the diagnosis of DAH. She was managed symptomatically, with full recovery. Conclusion: DAH is an uncommon, life-threatening, complication of dengue infection. A high index of suspicion, prompt evaluation and early institution of appropriate therapy hold the key to successfully manage such patients.

Diffuse Alveolar Haemorrhage: A Single Centre Experience

Introduction: Diffuse alveolar hemorrhage results from an accumulation of red blood cells into the alveolar space. Symptoms of alveolar hemorrhage are dyspnea, hemoptysis, anemia, diffuse pulmonary infiltrates and hypoxemic respiratory failure. Diagnosis is established by bronchoalveolar lavage and treatment includes a combination of high dose systemic corticosteroids, immunosuppressant and plasma exchange. The aim of this study is to evaluate the clinical radiological profile and laboratory findings and utility of bronchoalveolar lavage in the diagnosis of diffuse alveolar hemorrhage.Materials and Methods: In a retrospective review between February 2017 and December 2017, medical records of patients with a diagnosis of diffuse alveolar hemorrhage presenting at the National Academy of Medical Sciences, Kathmandu, Nepal, were analyzed. Clinical, radiology and laboratory results along with bronchoalveolar lavage results were extracted. Treatment received and clinical responses were evaluated.Results: A total of five patients were diagnosed to have diffuse alveolar hemorrhage based on bronchoalveolar lavage analysis. Three had hemorrhage secondary to Antineutrophil Cytoplasmic Antibody associated vasculitis, one had Systemic Lupus Erythematosus and the other Idiopathic Pulmonary Hemosiderosis. Renal involvement was present in three patients. All patients received systemic corticosteroids, three received Cyclophosphamide and one Rituximab for remission induction. Plasma exchange was done in two patients with severe hypoxemia. Of the five patients, four improved whereas one died.Conclusions: Diffuse alveolar hemorrhage presents with non-specific symptoms. Bronchoalveolar lavage is extremely useful to establish the diagnosis and exclude infections. Early initiation of immunosuppressant prevents respiratory failure and death.

DIFFUSE ALVEOLAR HEMORRHAGE IN PATIENTS WITH PRIMARY VS SECONDARY ANTIPHOSPHOLIPID ANTIBODY SYNDROME

SESSION TITLE: Diffuse Lung Disease SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2018 01:00 pm - 02:00 pm PURPOSE: We sought to determine the differences in clinical presentation, management and outcomes of diffuse alveolar hemorrhage in patients with primary and secondary antiphospholipid antibody syndrome (APS) METHODS: We conducted a retrospective chart review of 31 patients with diffuse alveolar hemorrhage (DAH) concurrent with primary and secondary antiphospholipid syndrome from January 1997 to January 2018. The diagnosis of DAH was achieved by i) bronchoalveolar lavage(BAL) revealing grossly hemorrhagic fluid from multiple lobes or increasingly hemorrhagic fluid return on BAL ii) >20% hemosiderin macrophages in BAL iii) DAH confirmed on surgical lung biopsy or autopsy. We reviewed their clinical presentations, laboratory, radiographic, bronchoscopic and pathologic findings and their clinical course. RESULTS: The baseline characteristics were as follows: median age 55 (range 25-88) years; predominantly male (58%); 19.3% smokers. Patients with primary APS (n = 19) and secondary APS (n =12) had similar demographic and laboratory profiles, with the exceptions of 95% of the patients with primary APS had anticardiolipin antibody (n=18) and 63% of them had one of the subtypes of beta-2 glycoprotein antibody (n=12) whilst 75% of the secondary APS patients had a positive anticardiolipin antibody positive(n=9) and 50% of those with a positive beta-2 glycoprotein antibody(n=6). Amongst those who underwent lung biopsies (n=9), 7 patients with primary APS had capillaritis versus only one of the secondary APS patients had capillaritis.DAH was first presentation in 21% (n=4) in primary APS but only in one case (8%) in secondary APS. The management of initial episode of DAH in secondary APS included: 67% required non-invasive ventilation (n=8) and 67% mechanical ventilation (n=8); 41% plasmapheresis (n=5) and 17 % ECMO (n=2). Only 15% of primary APS patients required non-invasive ventilation (n=3), 31% mechanical ventilation(n=6); 21% needed multiple plasmapheresis (n=4) and no patient required ECMO. Despite aggressive management, similar percentage of patients had recurrence of DAH in both subsets with 68% in primary (n=13) and 67% in secondary (n=8) APS. DAH (n=2) and unevaluated sudden onset of respiratory failure (n=1) was the cause of death in primary APS whereas no patients died of DAH or respiratory failure in secondary APS at our institution. CONCLUSIONS: Anticardiolipin antibody is the most common antibody present in primary APS patients. Capillaritis is seen more commonly in primary APS as compared to secondary APS. More aggressive respiratory support was needed in DAH cases in the setting of secondary APS. CLINICAL IMPLICATIONS: DAH in the setting of APS, especially secondary APS can be severe. Prompt recognition and early aggressive treatment are needed. DISCLOSURES: No relevant relationships by Misbah Baqir, source=Web Response No relevant relationships by Jay Ryu, source=Web Response No relevant relationships by Swathi Sangli, source=Web Response No relevant relationships by Ting Yang, source=Web Response

Alveolar Hemorrhage in Vasculitis (Primary and Secondary).

Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Bronchoalveolar lavage, retrieving bright red fluid, is the best diagnostic clue, also excluding infection and other causes of hemoptysis. Although not recommended, lung biopsy is the gold standard for the diagnosis of DAH and pulmonary capillaritis. Pulmonary capillaritis may be primary as in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis or secondary to drugs (especially antithyroid drugs such as propylthiouracil), infections, connective tissue diseases especially systemic lupus erythematosus, or other small vessel vasculitides. Newer toxic causes of drugs of abuse may be difficult to diagnose. Granulomatosis with polyangiitis and microscopic polyangiitis are the most common causes of capillaritis and DAH, whereas DAH is extremely rare in eosinophilic granulomatosis with polyangiitis. When pulmonary capillaritis is not secondary to underlying systemic vasculitis, idiopathic pauci-immune pulmonary capillaritis may be considered, with or without ANCA. Supportive treatment strategy is mandatory in all cases of DAH. Mechanical ventilation and extracorporeal membrane oxygenation may be used in severe cases. Early identification and removal of the putative drug is crucial in drug-induced vasculitis/DAH and may obviate the need for immunosuppressive therapy. High-dose corticosteroids, intravenous cyclophosphamide, and recently rituximab are the mainstay of treatment in vasculitis. Plasma exchange is recommended in anti-glomerular basement membrane disease and in severe DAH associated with systemic lupus erythematosus and is used in selected cases in ANCA-associated vasculitis.

A Rare Case of Calcific Rheumatoid Constrictive Pericarditis Complicated by Diffuse Alveolar Hemorrhage and MPO-ANCA-Associated Systemic Vasculitis With Pauci-Immune Glomerulonephritis

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Diffuse alveolar hemorrhage(DAH) is a rare complication in patients with Rheumatoid arthritis(RA). The added calcific constrictive pericarditis, systemic vasculitis and pauci-immune glomerulonephritis(PIGN) in the same patient poses a unique diagnostic and therapeutic dilemma CASE PRESENTATION: A 60-year-old male with a history of RA, calcific constrictive pericarditis was admitted with fever, chills, dry cough and worsening exertional dyspena. He had diminished breath sounds in bilateral lower lung fields on exam. Computed tomography of the chest revealed patchy nodular infiltrate in the left lung and confluent ground glass infiltrate in the right lung with persistent pericardial calcifications. He developed worsening hemoglobin drop and severe hypoxemic respiratory failure worsening and was intubated. Bronchoscopy with sequential bronchoalveolar lavage was progressively more hemorrhagic and hemosiderin laden macrophages confirmed the diagnosis of DAH. He was started on intravenous methylprednisolone. A workup revealed high titers of antibodies (Table1). Due to severity of his illness he had serial plasmapheresis followed by treatment with intravenous cyclophosphamide. Renal biopsy showed PIGN. By hospital day 20 his respiratory and renal function improved and was successfully extubated DISCUSSION: We report for the first time to the best of our knowledge a rare case with chronic rheumatoid constrictive pericarditis complicated by DAH in the setting of MPO-ANCA associated vasculitis and PIGN. One of the rare cardiac complications of RA is constrictive pericarditis confirmed here by cardiac catheterization with equalization of diastolic pressures and discordant right and left ventricular pressures and pericardial calcifications on imaging. DAH complicates 40% of cases of MPO-ANCA vasculitis however, limited data is available in adults with concurrent RA and is associated with higher mortality. The incidence of ANCA in RA is around 40% and both p- and c-ANCA staining patterns are expressed. one study described three RA patients with renal manifestations who improved with a combination of plasmapheresis and cyclophosphamide with or without methylprednisolone such as in our patient. CONCLUSIONS: A high index of suspicion, careful analysis and early detection is essential to improve chances of recovery in these patients Reference #1: Gauhar UA et al. Pulmonary manifestations of rheumatoid arthritis.Semin RespirCrit Care Med 2007;28:430-40. Reference #2: Kurita N et al. MPO-ANCA glomerulonephritis with RA.ClinExpNephrol.2010Aug;14(4):325-32. DISCLOSURE: The following authors have nothing to disclose: Kavitha Gopalratnam, Despina Michailidou, Ashley Rosario-Durchhalter, Paul Cohen, Armand Wolff No Product/Research Disclosure Information

Pulmonary Hemorrhage Secondary to Disseminated Strongyloidiasis in a Patient with Systemic Lupus Erythematosus

Introduction. Pulmonary hemorrhage secondary to disseminated strongyloidiasis is an unusual, well-recognized entity in immunocompromised patients with autoimmune disease, which is associated with the hyperinfection syndrome, sepsis, and a high mortality rate. Case Presentation. We present a case of a 44-year-old Mexican woman with systemic lupus erythematosus and acute bacterial meningitis who developed pulmonary hemorrhage with acute respiratory failure requiring mechanical ventilation, treated with broad spectrum systemic antibiotics and high dose methylprednisolone, who subsequently developed a characteristic purpuric skin eruption and septic shock and died two days later of refractory hypoxemia caused by massive pulmonary bleeding. The postmortem examination reports filariform larvae of S. stercolaris in lung, skin, and other organs. Conclusion. This case highlights the importance of considering disseminated strongyloidiasis in the differential diagnosis of diffuse alveolar hemorrhage in systemic lupus erythematosus, and screening for S. stercolaris infection before initiation of immunosuppressive therapy should be considered, especially in endemic areas. Disseminated strongyloidiasis has a high mortality rate, explained in part by absence of clinical suspicion.

A Rare Case of Diffuse Alveolar Hemorrhage Secondary to Acute Pulmonary Histoplasmosis.

Diffuse alveolar hemorrhage (DAH) is a rare presentation of acute pulmonary histoplasmosis. While histoplasmosis has been reported to cause hemoptysis and alveolar hemorrhage in children, the English language literature lacks any adult case reports documenting this association. We report a case of pulmonary histoplasmosis where the initial presentation was pneumonia with a subsequent diagnosis of DAH.

Idiopathic pulmonary hemosiderosis presenting in an adult: A case report and review of the literature.

Diffuse alveolar hemorrhage (DAH) is characterized by the presence of hemoptysis, anemia, and the presence of diffuse parenchymal infiltrates on imaging studies. Idiopathic pulmonary hemosiderosis (IPH) is an uncommon cause of diffuse alveolar hemorrhage (DAH) and is classically known to present in childhood. Adult-onset IPH is extremely rare. We report the case of a 48-year-old female patient who presented with hemoptysis and acute hypoxic respiratory failure, requiring intubation and mechanical ventilation. Imaging studies showed diffuse bilateral patchy infiltrates. Bronchoalveolar lavage (BAL) confirmed the diagnosis of DAH. Extensive workup including video-assisted thoracoscopic surgical lung biopsy (VATS) failed to reveal any vasculitis, infectious, immunological or connective tissue disorder, as the underlying cause for DAH. The patient was successfully treated with high-dose steroid therapy.

Immune Diffuse Alveolar Hemorrhage: A Retrospective Assessment of a Diagnostic Scale

Initiating early steroid treatment in patients with immune diffuse alveolar hemorrhage (DAH) is a key aspect of early management. However, steroid initiation is often delayed until the results of immunological markers and/or tissue biopsy have been obtained, which could contribute to poor outcomes. We previously developed a clinical score allowing for the early diagnosis of DAH of immune causes. However, this score has not been validated in an independent cohort of patients. The aim of this study was to assess the validity of this diagnostic score using an independent cohort of patients admitted for DAH of immune and nonimmune causes. We conducted a retrospective cohort study of patients admitted between January 2002 and December 2009 for DAH of immune and nonimmune causes. Forty-six patients were included in the study, with 12 patients having immune DAH and 34 patients with nonimmune DAH. Application of our previously validated clinical scale of immune DAH to this independent population of patients yielded an area under the ROC curve of 0.95 [0.90-1.01]. A score ≥4/10 was associated with the best performances of this scale: sensitivity=1.00 [0.73-1.00], specificity=0.88 [0.72-0.97], positive predictive value=0.75 [0.48-0.93], and negative predictive value=1.00 [0.88-1.00]. While immunological tests and tissue biopsy results are pending, deciding whether to initiate an immunosuppressive treatment is challenging. The initiation of early corticosteroid treatment is warranted in patients with immune DAH and could improve outcomes. This study confirms that this score allows for a good discrimination between patients with immune and nonimmune DAH. Because this series has several limitations, including its single-center and retrospective nature, the small number of patients included, and the lack of therapeutic intervention, a prospective evaluation of this score is warranted to ascertain whether it can improve the adequacy of early treatment strategies and thus improve the outcomes of DAH patients.

Alveolar hemorrhage and kidney disease: Characteristics and therapy

Anti-neutrophil cytoplasmic antibody-associated vasculitis and Goodpasture's glomerular basement membrane disease are the most common causes of diffuse alveolar hemorrhage, a life-threatening disease. Systemic lupus erythematosus and the antiphospholipid syndrome are also causes of alveolar hemorrhage. We retrospectively reviewed 15 cases of diffuse alveolar hemorrhage (DAH) associated with renal diseases. Diagnosis of DAH was based on the presence of bloody bronchoalveolar lavage fluid. There were three men and 12 women, with a mean age of 50.5 years (extremes: 24-74 years). Proteinuria and hematuria were observed, respectively, in 15 and 14 cases. Six patients revealed arterial hypertension. Crescentic glomerulonephritis was diagnosed with kidney biopsies in ten cases. The etiology of renal disease was microscopic polyangiitis (MPA) in seven cases, Wegener disease in four cases, systemic lupus erythematous in one case, cryoglobulinemia in one case, myeloma in one case and propyl-thiouracil-induced MPA in one case. Hemoptysis occurred in 14 cases. The mean serum level of hemoglobin was 7.1 g/dL (5.1-10 g/dL). The mean serum creatinine concentration was 7.07 mg/dL (2.4-13.7 mg/dL). Gas exchange was severely compromised, with an oxygenation index <80 mmHg in 14 patients and <60 mmHg in seven patients. Bronchoalveolar lavage was performed in 11 cases, and had positive findings for hemorrhage in all. Methylprednisolone pulses and cyclophosphamide were used in 14 patients. Plasmapheresis was performed in three cases. One patient received cycles of Dexamethasome-Melphalan. Three patients died as a result of DAH. The mortality rate in our study was 20%.

Diffuse Alveolar Hemorrhage

Diffuse alveolar hemorrhage (DAH) is a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as anti-neutrophil cytoplasmic antibody-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus, but DAH may also result from coagulation disorders, drugs, inhaled toxins, or transplantation. The diagnosis of DAH relies on clinical suspicion combined with laboratory, radiologic, and pathologic findings. Early recognition is crucial, because prompt diagnosis and treatment is necessary for survival. Corticosteroids and immunosuppressive agents remain the gold standard. In patients with DAH, biopsy of involved sites can help to identify the cause and to direct therapy. This article aims to provide a general review of the causes and clinical presentation of DAH and to recommend a diagnostic approach and a management plan for the most common causes.

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Introduction: Diffuse alveolar hemorrhage (DAH) is a clinical syndrome that can manifest as a complication in cancer patients and carries a substantial mortality. Minimal data exists regarding effective treatment options. Retrospective studies have cited high-dose steroids as primary therapy with fibrinolytic agents used adjunctively may reduce mortality. Practices at our institution have changed to include the use of fibrinolytic agents in addition to steroids for the acute treatment of DAH. Hypothesis: This study was conducted to characterize current prescribing practices at a comprehensive cancer center for the acute treatment of DAH in critically ill cancer patients and to determine the impact of steroid dosing on mortality in patients receiving methylprednisolone (MP) with aminocaproic acid (ACA) infusions. Methods: Medical intensive care unit patients > 18 years of age with a diagnosis of DAH treated with MP and ACA were retrospectively analyzed over 48 consecutive months. Diagnosis was based on clinical presentation consistent with DAH or available bronchoscopic and autopsy findings. Data collection included patient demographics, pharmacological treatment, ventilator days, ICU length of stay, thrombotic complications, bronchoscopic and autopsy findings, and mortality. Low, medium, and high-dose MP was defined as less than 250 mg, 250 to less than 1000mg, or greater than or equal to 1000 mg, respectively. Results: 206 cancer patients were identified who received MP at a mean dose of 502 mg/day and ACA infusion at 1 g/hour. There were no differences in baseline characteristics including age, platelet count, oxygen requirements, or APACHE II and SOFA scores. 55 patients received low-dose MP, 105 medium-dose MP, and 46 high-dose MP. Amongst the 3 groups, there were no differences in 100-day mortality (85.5%, 78.1%, 91.3%, p=0.12). There were also no differences in ventilator days (p=0.12), ICU length of stay (p=0.79), or hospital length of stay (p=0.82). Conclusions: DAH is a significant contributor to mortality in the cancer population. In patients treated with MP and ACA, escalation of MP dose may have no improvement on outcomes and survival in this patient population.

Diffuse Alveolar Hemorrhage and Acute Respiratory Distress Syndrome During Treatment of Rheumatoid Arthritis With Etanercept

Etanercept (Enbrel) is a drug targeted against tumor necrosis factor-α, which is increasingly being used in treatment of a variety of autoimmune disorders. It has been reported to have a relatively safe profile; reported reactions have been of minor to moderate severity and range from hypersensitivity reactions, serious infections, fulminant pneumonias, and lung nodules. We report a case of diffuse alveolar hemorrhage and acute lung injury probably related to its use in a patient with rheumatoid arthritis. A high index of suspicion for noninfectious pulmonary diseases should also be considered when a patient with autoimmune disease treated with etanercept presents with pulmonary infiltrates and hypoxia refractory to antibiotics. Flexible bronchoscopy with sequential lavage should be attempted to detect and confirm the diagnosis of diffuse alveolar hemorrhage even if the patient does not have hemoptysis.