Severe venous thrombosis in granulomatosis with polyangiitis (Wegener's)

Sir, Veins are seldom involved in systemic vasculitis with the notable exception of Behcet’s disease. We recently encountered severe venous thrombosis in a newly diagnosed case of classical Granulomatosis with Polyangiitis i.e. GPA (Wegener’s). This 32-year old male presented in February 2010 with polyarthralgia for 2 months along with epistaxis, cough and redness in eyes for one month. Physical examination was normal except for bilateral conjunctivitis and mild bilateral conductive deafness. Laboratory tests showed Hb 14.8 g/dl, WBC 9000/cu mm, N62L23, platelets 386,000/cu mm, ESR 77 mm, CRP 44.4 mg/l, urea 21 mg/dl, creatinine 0.9 mg/dl, urine analysis 20e25 RBC/hpf, protein and sugar nil, rheumatoid factor negative, ANA negative, c-ANCA positive (immunofluorescence) and anti-proteinase 3 strongly positive (>100 U/ml). Chest X-ray showed multiple nodular and infiltrative lesions in both lungs (Fig. 1). With the above features, a diagnosis of GPA (Wegener’s) was made and patient was started on oral prednisolone 40 mg/day along with calcium, vitamin D and alendronate. An intravenous infusion of cyclophosphamide 1000 mg was also administered. Patient returned after 8 days with acute pain and swelling of the left lower limb up to mid thigh along with cough and mild haemoptysis. Marked calf tenderness and a few palpable purpuric spots were noted on left leg. Colour doppler confirmed the presence of thrombosis in great saphenous vein extending partially into femoral vein. Lab results showed Hb 10.9 g/ dl, WBC 16,300/cu mm with 85% neutrophils, platelets 590,000/cu mm, urea 26 mg/dl, creatinine 0.9 mg/dl, urine microscopy 80 RBC, 8e10 WBCs and 3e4 granular casts/ hpf, 24-h urinary protein 3.2 g, serum protein 5.8 g/dl, albumin 2.8 g/dl, AST/ALT 48/75 units/ml. Antiphospholipid antibodies and lupus anticoagulant were negative. Patient was started on unfractionated heparin infusion along with oral warfarin, and given intravenous