Alveolar Hemorrhage in Vasculitis (Primary and Secondary)

Abstract

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.

Extracorporeal Membrane Oxygenation for Severe ARDS Due to Immune Diffuse Alveolar Hemorrhage

“…Diffuse alveolar hemorrhage (DAH) is a rare but lifethreatening disease that may arise from immune entities such as antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis and anti-glomerular basement membrane antibody disease. 1 Prognosis becomes poor once ICU admission is required, with a reported mortality up to 77% when mechanical ventilation is necessary. 2,3 Traditionally, active bleeding has been considered a contraindication for extracorporeal membrane oxygenation (ECMO) support in DAH, but some case reports (in total 16 patients) have reported feasibility in selected cases.…”

51-Year-Old Man With Eye Pain and a Cough

“…The most dreaded complications include DAH, which develops in 7% to 13% of cases, and fulminant renal failure secondary to rapidly progressive glomerulonephritis. 9 Our patient developed hypoxemia and respiratory failure secondary to DAH, which is usually diagnosed through bronchoalveolar lavage, revealing an increasing bloody return in each subsequent aliquot. 9 Although a lung biopsy is the diagnostic criterion standard, it is often not performed in critically ill patients.…”

“…9 Our patient developed hypoxemia and respiratory failure secondary to DAH, which is usually diagnosed through bronchoalveolar lavage, revealing an increasing bloody return in each subsequent aliquot. 9 Although a lung biopsy is the diagnostic criterion standard, it is often not performed in critically ill patients. When DAH is present, the causative factor needs to be identified.…”

Clinical profile of patients with systemic autoimmune diseases treated in the intensive care unit who developed diffuse alveolar haemorrhage – an observational retrospective cohort study

“…Diagnosis of DAH is complex and poses a par ticular challenge in critically ill patients. The typical clinical symptom -hemoptysis -is present only in approximately one third of cases [1,4]. The most cru cial imaging method is chest computed tomography (CT), which most often reveals ground glass opacities and/or alveolar consolidation with air bronchogram.…”

“…One of the most typical features of DAH is increased diffusion lung capacity for carbon monoxide (DLCO) in lung func tion tests. The gold standard for the diagnosis of DAH is, however, surgical biopsy [4]. Obviously, in the ICU setting, some of the aforementioned methods pose too high a threat to patients.…”