Description
The approach to small vessel vasculitis involves a comprehensive assessment, including clinical evaluation, laboratory tests, and imaging studies to confirm the diagnosis and identify underlying causes. Treatment typically focuses on immunosuppressive therapies, such as corticosteroids and cytotoxic agents, to control inflammation and prevent organ damage. In some cases, targeted biologic therapies may be considered. Early detection and intervention are crucial to prevent complications and improve long-term outcomes.
Summary Listen
- Vasculitis is a disorder characterized by inflammation and necrosis of blood vessels, leading to impaired blood flow and organ dysfunction. It can be immune-mediated or caused by direct invasion of infectious pathogens. Histopathology reveals transmural inflammatory infiltrate and thrombus formation within the vessel walls.
- The classification of vasculitis depends on the size of the affected vessels: large, medium, or small. Large vessel vasculitis involves the aorta and its main branches; medium vessel vasculitis affects branches beyond the main origin until they lose their muscular coat; and small vessel vasculitis involves terminal arterioles, capillaries, and venules. There can be overlap between the three types of vasculitis.
- Clinical features vary depending on the type of vasculitis. Large vessel vasculitis presents with symptoms like claudication, vision loss, and headache. Medium vessel vasculitis shows cutaneous ulcers, nodules, renal vascular hypertension, and mononeuritis multiplex. Small vessel vasculitis manifests as palpable purpura, hemoptysis, hematuria, splinter hemorrhages, and scleritis.
- Small vessel vasculitis includes ANCA-associated vasculitis (MPA, GPA, EGPA) and immune complex-mediated vasculitis (anti-GBM disease, cryoglobulinemic vasculitis, IgA vasculitis). Variable vessel vasculitis includes Behcet's disease and Cogan syndrome. Vasculitis can also be organ-limited or associated with autoimmune conditions, specific etiologies (hepatitis B/C, syphilis), drug use, or cancer.
- GPA diagnosis criteria include nasal bloody discharge, cartilage involvement, and inflammatory hearing loss. Clinical features include palatal perforation, saddle nose deformity, epistaxis, DAH, and rapidly progressive renal failure. Management involves induction therapy (rituximab or cyclophosphamide with steroids) and maintenance therapy (rituximab or azathioprine).
- MPA classification criteria involve the absence of upper respiratory tract involvement, PR3 positivity, and high eosinophil count. Clinical features include constitutional symptoms, renal involvement, DAH, pulmonary fibrosis, vasculitic neuropathy, and skin symptoms. EGPA is characterized by late-onset asthma, upper respiratory tract involvement, neurological involvement, and eosinophilia.
- Severe vasculitis manifestations include diffuse alveolar hemorrhage, rapidly progressive renal failure, multiple cranial nerve palsies, CNS vasculitis, mononeuritis multiplex, cardiac involvement, and mesenteric or limb ischemia. ANCA testing should be considered in cases of glomerulonephritis, pulmonary hemorrhage, cutaneous vasculitis, multiple lung nodules, destructive upper airway disease, mononeuritis multiplex, and ocular symptoms.
- Cryoglobulinemic vasculitis can be mixed cryoglobulinemia or type 2, associated with hepatitis C, hepatitis B, and lymphoproliferative disorders. Diagnosis requires a combination of questionnaire, clinical, and laboratory items, including constitutional symptoms, articular involvement, vasculitic involvement, reduced C4 levels, positive rheumatoid factor, and positive serum M component. Treatment involves steroids, plasmapheresis, cyclophosphamide, and rituximab.
- It's essential to rule out vasculitis mimics, including infections, malignancies, atrial myxomas, fibromuscular dysplasia, Buerger's disease, infective endocarditis, antiphospholipid syndrome, HCV, HIV, and sepsis. Awareness of potential vasculitis symptoms and early referral to rheumatology are crucial for prompt diagnosis and management.
- Diabetic patients with vasculitis should be treated similarly to other patients, with careful control of diabetes and potentially lower doses of corticosteroids. The most common complications in MPA are renal damage, pulmonary fibrosis, and nerve damage, highlighting the importance of early and timely treatment.
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About the Speakers
Dr. Kriti Kishor
Senior Consultant Rheumatology, Yatharth Super Speciality Hospital, Noida
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