Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).

摘要: Microscopic polyangiitis (MPA) is a systemic small-vessel vasculitis primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. In this retrospective study of 29 patients MPA alveolar hemorrhage (AH), we characterized the manifestations at presentation assessed short- long-term outcome. AH was diagnosed when bronchoalveolar lavage macroscopically bloody, or contained hemosiderin-laden macrophages, in absence lung infection edema. focal segmental kidney biopsy pathologically proved vasculitis. There were 17 women 12 men, mean age 55.8 +/- 16.7 years. The onset rapidly progressive, but 8 (28%) patients, symptoms preceded diagnosis for more than 1 year. most constant findings 28 (97%) patients; fever (62%); myalgia arthralgia (52%); weight loss (45%); ear, nose, throat (31%); skin involvement (17%). Lung opacities bilateral 26 (90%) frequently involving lower part lungs. Bronchoalveolar lavage, performed 27 hemorrhagic 25 (93%), numerous siderophages others. Most severely anemic (mean hemoglobin, 8.1 1.8 g/dL). ANCA, present (93%) gave perinuclear (14), cytoplasmic (11), mixed (1) pattern. Mean serum creatinine level 407 415 mumol/L. Renal confirmed presence patients. Patients treated corticosteroids (100%), cyclophosphamide (79%), plasmapheresis (24%), dialysis (28%), mechanical ventilation (10%). overall mortality rate 31% (9 patients). Deaths related to (5 patients) side effects treatment (4). frequent aged mechanically ventilated 5-year survival 68%. recovery respiratory function among survivors clinically considered complete 20 (69%) However, 7 (24%) had persistent alterations on tests. Of 11 who relapses, 2 died from AH.