Diffuse alveolar hemorrhage resulting from Pauci-immune pulmonary capillaritis

Monteiro, Andreia Salarini; Addor, Gabriela; Nigri, David Henrique; Torres, Wilhermo; Franco, Carlos Alberto de Barros

doi:10.1590/s0102-35862003000400010

Cited by 4 publications

(4 citation statements)

References 8 publications

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“…Follow-up is recommended to ascertain that systemic signs do not develop subsequently. 1,[53][54][55] Glucocorticoids associated with intravenous (IV) cyclophosphamide have been adopted as an accepted treatment strategy. 53,56 Rituximab, a monoclonal anti-CD20 antibody, has been used as rescue therapy when cyclophosphamide fails.…”

Section: Isolated Pauci-immune Pulmonary Capillaritismentioning

confidence: 99%

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Nasser

Cottin

2018

Semin Respir Crit Care Med

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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.

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Section: Isolated Pauci-immune Pulmonary Capillaritismentioning

confidence: 99%

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Nasser

Cottin

2018

Semin Respir Crit Care Med

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“…In 1997, Jennings et al described pauci-immune pulmonary capillaritis in eight out of twentynine patients with DAH and biopsy proven pulmonary capillaritis. We performed a search on PubMed and MEDLINE on key words of "pauci immune pulmonary capillaritis" and "di use alveolar hemorrhage" and identi ed eight case reports where pauci-immune pulmonary capillaritis resulted in DAH [4][5][6][7][8][9][10][11] and one where there was no evidence of DAH, only neutrophils in the interalveolar interstitium on histology [12].…”

Section: Discussionmentioning

confidence: 99%

Isolated Pauci-Immune Pulmonary Capillaritis Associated with Hydrocarbon Inhalation and Marijuana Smoking: An Unusual Case of Severe Hypoxemia

Wong

Bajaj

et al. 2020

Case Reports in Pulmonology

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We present a case report of a patient with Isolated pauci-immune pulmonary capillaritis (IPIPC). A 40-year-old male presented with acute onset severe hypoxemic respiratory failure. He had just returned home from work as a cabinetmaker, where he experienced inhalational exposure to hydrocarbons and solvents, and had smoked a marijuana cigarette. He was hypotensive, and his chest imaging showed bilateral dependent infiltrates. His hypoxemia made little improvement after conventional ventilator support and broad-spectrum antibacterial therapy and he was considered too unstable to tolerate diagnostic bronchoscopy with bronchoalveolar lavage. His laboratory evaluation initially showed microscopic hematuria which later cleared, but other tests including serologic autoimmune assessment were negative, and he did not have any traditional risk factors for vasculitis. A video-assisted thoracoscopic lung biopsy revealed diffuse alveolar hemorrhage with pulmonary capillaritis on histopathology. He was diagnosed with IPIPC and initiated on immunosuppressive therapy. He was soon liberated from mechanical ventilation and improved to hospital discharge. Diffuse alveolar hemorrhage from Goodpasture’s Syndrome has manifested following inhalation of hydrocarbons and following smoking. This has not previously been reported with IPIPC. Given the lack of other findings and risk factors, his IPIPC was likely associated with occupational exposures to hydrocarbons as a cabinetmaker compounded by marijuana smoking.

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“…Monteiro et al . [ 4 ] reported a case of IPIPC in a 27-year-old female. This patient used to consume cocaine and presented with hemoptysis and progressive dyspnea of short duration.…”

Section: Discussionmentioning

confidence: 99%

“…[ 3 ] Since then only sporadic case reports of the disorder are available in the English literature. [ 4 ] We are reporting such a case which, to the best of our knowledge and belief, is the first case report of the disorder in Indian medical literature.…”

Section: Introductionmentioning

confidence: 97%

Isolated pauci-immune pulmonary capillaritis

et al. 2015

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A young house wife presented with low grade fever, cough, haemoptysis and SOB of unknown aetiology for 40 days duration. Respiratory system examination revealed diffuse crepts and rhonchi. Other organ system examination did not reveal any abnormality. X-ray chest PA view and CT thorax showed diffuse bilateral necrotising nodular lesions of various sizes with small pleural effusion. She also had low resting oxygen saturation with falling haematocrit. Her Serum was week positive for p-ANCA and negative for MPO-ANCA. Bronchoscopy revealed continuous bloody aspirates. We could not isolate any organisms in any of the specimens from her and she was unresponsive to any of the antibiotics either. Based on the clinical, laboratory data, radiological features and positive outcome to pulse therapy of methylprednisolone and cyclophosphamide, she was diagnosed as a case of IPIPC.

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Diffuse alveolar hemorrhage resulting from Pauci-immune pulmonary capillaritis

Cited by 4 publications

References 8 publications

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Isolated Pauci-Immune Pulmonary Capillaritis Associated with Hydrocarbon Inhalation and Marijuana Smoking: An Unusual Case of Severe Hypoxemia

Isolated pauci-immune pulmonary capillaritis

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