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Lupus
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Wegener‘s granulomatosis associated with antiphospholipid syndrome

G Castellino

Divisione di Reumatologia, Azienda Ospedaliera Arcispedale Sant‘ Anna, Ferrara, Italy; Azienda Ospedaliera Arcispedale St‘ Anna, Divisione di Reumatologia, Corso Giovecca 203, 44100 Ferrara, Italy Tel: (+ 39) 0532 236314/236382; Fax: (+ 39) 0532 211946 g.castellino{at}usa.net

R La Corte

D Santilli

F Trotta

Divisione di Reumatologia, Azienda Ospedaliera Arcispedale Sant‘ Anna, Ferrara, Italy

We report a case of Wegener‘s granulomatosis (WG) with pulmonary hemorrhage also satisfying the criteria for antiphospholipid antibody syndrome (APS).

This association has, to the best of our knowledge, never been described before. Pulmonary hemorrhage may be an early manifestation of several immune and idiopathic disorders such as ANCA-associated vasculitis. Several case-reports of APS patients with capillaritis have been described. A possible explanation is that microvascular thrombosis with subsequent increase in vascular permeability facilitates perivascular IgG and complement deposition leading to development of capillaritis. Whether the vascular disease is secondary to thrombosis or vasculitis or both is important in choosing the proper management strategy. We suggest that anticardiolipin antibodies (aCL) should be detected in ANCA-associated vascularitis because they may contribute to life-threatening events superimposed on vascular damage.

Key Words: vasculitis • ANCA • lung • antiphospholipid syndrome

Lupus, Vol. 9, No. 9, 717-720 (2000)
DOI: 10.1191/096120300675018039
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