Adult Rheumatoid Vasculitis

Rheumatoid vasculitis is systemic small and medium vessel necrotising vasculitis complicating long standing, severe, seropositive rheumatoid arthritis.

Now rare (under 1%) with modern DMARD therapy.

Risk factors: male sex, high titre rheumatoid factor and anti-CCP, subcutaneous nodules, smoking, long disease duration (over 10 years), previous joint replacement.

Pathology: immune complex deposition with leukocytoclastic or necrotising arteritis.

Mortality 30 to 40% at 5 years with severe visceral disease.

• Digital infarcts, nailfold lesions, palpable purpura, livedo, cutaneous ulcers (often at malleoli).

• Mononeuritis multiplex (foot drop, wrist drop), sensory neuropathy.

• Scleritis and peripheral ulcerative keratitis, pericarditis, pleuritis, mesenteric ischaemia, myocardial ischaemia, renal involvement (rare).

• Systemic features: fever, weight loss, high ESR.

• High titre rheumatoid factor and anti-CCP, low C3/C4 (consumption), cryoglobulins, raised ESR/CRP.

• Biopsy of skin or affected nerve/muscle: necrotising vasculitis.

• Exclude infection, ANCA vasculitis (ANCA, MPO/PR3), drug-induced vasculitis, cryoglobulinaemia, hepatitis B/C.

• Angiography for visceral involvement; nerve conduction studies; urinalysis, creatinine; echocardiography.

• Ophthalmology review if eye symptoms.

• Severe or visceral disease: methylprednisolone 500 to 1000 mg IV daily for 3 days, then prednisolone 1 mg/kg/day tapered; with cyclophosphamide 15 mg/kg IV every 2 to 3 weeks for 6 pulses (or 2 mg/kg orally) or rituximab 1 g on days 1 and 15 (preferred over cyclophosphamide in many).

• Maintenance: azathioprine 2 mg/kg or methotrexate after remission; continue appropriate rheumatoid arthritis DMARD therapy.

• Limited cutaneous disease: prednisolone 20 to 40 mg with methotrexate or azathioprine; local wound care.

• Digital ischaemia: nifedipine 30 to 60 mg daily, aspirin 75 mg, avoid smoking, consider iloprost.

• Scleritis: topical and systemic steroids, rituximab for refractory cases.

• Stop TNF inhibitors if implicated.

• Pneumocystis prophylaxis, bone protection, monitoring for infection.