Antigen inhalation as a triggering factor in systemic small-sized-vessel vasculitis. Four cases.
In order to identify small-sized-vessel vasculitis occurring immediately after massive inhalation of particles which could be considered antigenic, we performed a retrospective review of patients who consulted our Department of Internal Medicine between 1980 and 1998 and were diagnosed as having small-vessel vasculitis that developed immediately after massive exposure to inhaled particles. Four patients, 27 to 55 years old, presented with small-sized-vessel vasculitis (one with Wegener's granulomatosis and three with Churg-Strauss syndrome) which developed after massive inhalation of particles. Clinical manifestations occurred from a few hours to 10 days after inhalation of diesel fumes, cereal dust, flour or cereal dust and/or pigeon droppings. Three patients remain disease-free 8, 9 and 18 years after discontinuing treatment. One patient remains well after 3 years on low-dose corticosteroids and cyclophosphamide therapy. Our data suggest that primary antigens can cause small-sized-vessel vasculitides. Identifying such etiological factors could improve our understanding of the pathogenesis of systemic vasculitides and prevent relapses due to similar antigenic exposure.
Mouthon
L
L
Service de Médecine Interne, Hôpital Avicenne, Université Paris-Nord, Bobigny, Cedex, France. luc.mouthon@avc.ap-hop-paris.fr
Khaled
M
M
Cohen
P
P
Subra
J F
JF
Guillevin
L
L
D000328:Adult; D000818:Animals; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000941:Antigens / Q000009:adverse effects*; D015267:Churg-Strauss Syndrome / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D010856:Columbidae; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004391:Dust / Q000009:adverse effects*; D004417:Dyspnea / Q000209:etiology; D002523:Edible Grain / Q000009:adverse effects*; D005243:Feces; D005260:Female; D005334:Fever / Q000209:etiology; D005433:Flour; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D019570:Inhalation Exposure / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D012189:Retrospective Studies; D012307:Risk Factors; D012882:Skin Tests; D013256:Steroids; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D001335:Vehicle Emissions / Q000009:adverse effects*
D000893:Anti-Inflammatory Agents; D000941:Antigens; D004391:Dust; D007166:Immunosuppressive Agents; D013256:Steroids; D001335:Vehicle Emissions; D003520:Cyclophosphamide