Pulmonary Capillaritis

Lee, Augustine S.; Specks, Ulrich

doi:10.1055/s-2004-836146

Cited by 20 publications

(17 citation statements)

References 49 publications

(78 reference statements)

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“…Immunemediated mechanisms can cause DAH with or without the presence of capillaritis (2). The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) syndromes, granulomatosis with polyangiitis (Wegener's) (GPA) and microscopic polyangiitis (MPA), are the most common cause of pulmonary capillaritis leading to DAH (2)(3)(4). DAH is a severe manifestation of AAV, commonly associated with glomerulonephritis and often leading to respiratory failure requiring ventilatory support in the intensive care unit (ICU).…”

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confidence: 99%

Diffuse Alveolar Hemorrhage Secondary to Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Predictors of Respiratory Failure and Clinical Outcomes

Cartin‐Ceba

Diaz-Caballero²,

Al-Qadi

et al. 2016

Arthritis & Rheumatology

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107

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Objective. To identify predictors of respiratory failure and to evaluate the therapeutic efficacy of plasma exchange (PE) and of rituximab versus cyclophosphamide in a cohort of patients with diffuse alveolar hemorrhage (DAH) secondary to antineutrophil cytoplasmic antibody-associated vasculitis (AAV) with or without respiratory failure.Methods. We performed a single-center historical cohort study of all consecutive patients with AAVassociated DAH who were evaluated over a 16-year period. Logistic regression models were developed to examine the predictive role of the baseline clinical characteristics for the development of respiratory failure, and for the effect of PE and remission induction therapy on the main outcome (complete remission at 6 months).Results. Seventy-three patients with DAH were identified, and 34 of them experienced respiratory failure. The degree of hypoxemia upon initial presentation, a higher percentage of neutrophils in the bronchoalveolar lavage fluid cell count, and higher C-reactive protein levels were independently associated with the development of respiratory failure. PE was not associated with achieving complete remission at 6 months, with an odds ratio (OR) of 0.49 (95% confidence interval [95% CI] 0.12-1.95) (P 5 0.32). Rituximab treatment was independently associated with achieving complete remission at 6 months (OR 6.45 [95% CI 1.78-29], P 5 0.003).Conclusion. Our findings indicate that the most important predictor of respiratory failure in patients with DAH secondary to AAV is the degree of hypoxemia upon presentation. No clear benefit of the addition of PE to standard remission induction therapy was demonstrated. Complete remission by 6 months was achieved at a higher rate with rituximab than with cyclophosphamide in patients with DAH secondary to AAV, including those needing mechanical ventilation.

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confidence: 99%

Diffuse Alveolar Hemorrhage Secondary to Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Predictors of Respiratory Failure and Clinical Outcomes

Cartin‐Ceba

Diaz-Caballero²,

Al-Qadi

et al. 2016

Arthritis & Rheumatology

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107

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Section: Introductionmentioning

confidence: 99%

“…In the acute phases, it is characterised by the presence of dyspnoea with hypoxaemia, haemoptysis (in 70% of cases) and sideropenic anaemia [1]. Anaemia is the most constant finding in patients with pulmonary haemorrhage and is present in virtually all patients with Goodpasture's syndrome [1]. Haemoptysis is the most striking symptom and may be present intermittently for weeks before the acute event or, more commonly, it may manifest or worsen dramatically and suddenly over a few days or even hours.…”

Section: Introductionmentioning

confidence: 99%

“…Diffuse alveolar haemorrhage (DAH) is a rare clinicopathological syndrome characterised by diffuse intra-alveolar bleeding, which may have an insidious chronic course or may present as an acute event in subjects of any age, including children [1,2]. DAH must be distinguished from diffuse alveolar aspiration of blood from pulmonary focal lesions (e.g.…”

Section: Introductionmentioning

confidence: 99%

“…L'emorragia alveolare diffusa (EAD) è una rara sindrome clinico-patologica caratterizzata da sanguinamento intraalveolare diffuso, che può avere un decorso insidioso e cronico o presentarsi come un evento acuto in soggetti di qualsiasi età, compresi pazienti in età pediatrica [1,2]. L'EAD deve essere distinta dall'aspirazione alveolare diffusa di sangue proveniente da lesioni focali del polmone (es.…”

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Radiological aspects of diffuse alveolar haemorrhage

et al. 2008

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Primary Antiphospholipid Syndrome–Associated Diffuse Alveolar Hemorrhage

Cartin‐Ceba

Peikert

Ashrani

et al. 2014

Arthritis Care & Research

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Objective. Diffuse alveolar hemorrhage (DAH) is an uncommon complication of primary antiphospholipid syndrome (APS). We aimed to describe the clinical characteristics, treatment, and outcomes of primary APS-associated DAH in a single center. Methods. We conducted a retrospective review of all adults with primary APS-associated DAH evaluated at Mayo Clinic over a 15-year period. DAH was defined as bilateral pulmonary infiltrates and bronchoalveolar lavage (BAL) fluid documenting progressively bloody returns and/or the presence of >20% hemosiderin-laden macrophages. Patients with other causes of DAH were excluded. Results. Eighteen patients were identified (median age 43 years). Capillaritis was present in surgical lung biopsy samples of 3 patients. BAL differential cell counts revealed predominantly neutrophils. All patients were treated initially with glucocorticoids. Cyclophosphamide (CYC) was used in 8 patients; complete remission was achieved in 3 patients treated with CYC alone and in 1 patient receiving combination therapy with rituximab (RTX). RTX was used in 9 patients; 2 patients achieved remission with RTX alone, whereas 3 patients required combination therapy with CYC or mycophenolate mofetil (MMF). No patient achieved complete remission while receiving single therapy with MMF, azathioprine, or plasma exchange. Intravenous gamma globulin therapy was administered in 5 patients; no patient achieved control of the disease. Six patients died, all because of complications related to uncontrolled DAH or its therapy. Conclusion. We present the largest case series of primary APS-associated DAH reported in the literature. DAH carries a very poor prognosis and therapeutic options are limited. Immunosuppression with either CYC or RTX is associated with the highest likelihood of remission induction and should be considered early.

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Pulmonary Capillaritis

Cited by 20 publications

References 49 publications

Diffuse Alveolar Hemorrhage Secondary to Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Predictors of Respiratory Failure and Clinical Outcomes

Diffuse Alveolar Hemorrhage Secondary to Antineutrophil Cytoplasmic Antibody–Associated Vasculitis: Predictors of Respiratory Failure and Clinical Outcomes

Radiological aspects of diffuse alveolar haemorrhage

Primary Antiphospholipid Syndrome–Associated Diffuse Alveolar Hemorrhage

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