Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions • Mononeuritis multiplex • Hematuria, proteinuria, rapidly progressing GN • New pulmonary and renal abnormalities SMALL VESSEL VASCULITIS: ANCA Associated - ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA): • Microscopic Polyangiitis: - No Granulomas - ANCA is present in >90%, MPO > PR3 - Necrotizing glomerulonephritis and/or pulmonary capillaritis • EGPA: - Eosinophilia, asthma, chronic rhinosinusitis, + Granulomas - MPO > PR3 60% • GPA: - +Granulomas - ANCA is present in >80% (80-90% have PR3-ANCA) - Necrotizing, pauci-immune glomerulonephritis - Granulomatous inflammation of the upper and lower respiratory tracts Immune Complex Vasculitis • Glomerulonephritis is often present • Henoch Schonlein Purpura (IgA vasculitis) - Affects the skin and gastrointestinal tract, and often causes arthritis. (± glomerulonephritis) • Anti-GBM - (10-40% ANCA +MPO) • Cryoglobulinemic Vasculitis - Skin, glomeruli, and peripheral nerves are often involved • Hypocomplementemic Urticarial Vasculitis (anti-C1q Vasculitis): - Urticaria and hypocomplementemia, Glomerulonephritis, arthritis, obstructive pulmonary disease, and ocular inflammation Others: • Lupus Vasculitis • Drug Associated (Commonly MPO-ANCA) • Levamisole Associated: (± 100 % Commonly MPO-ANCA) • Hydralazine-Induced ANCA-Associated Vasculitis: + MPO-ANCA MEDIUM VESSEL VASCULITIS: • Polyarteritis Nodosa: - Kidneys: glomerular ischemia - Skin: erythematous nodules, purpura, livedo reticularis, ulcers, and bullous or vesicular eruption - Joints: myalgia and muscle weakness - Nerves: neuropathy - GI tract: abdominal pain • Kawasaki Disease: - Mucocutaneous lymph node syndrome - Coronary arteries - Fever - Conjunctivitis, mucositis - Rash, arthritis LARGE VESSEL VASCULITIS (Granulomatous disease): • Takayasu: - Age < 30 years - Aorta and branches • GCA: - Aorta and Superficial temporal artery - Age > 50 years VARIABLE-VESSEL VASCULITIS: • Behçet Syndrome: - Recurrent oral and/or genital aphthous ulcers, cutaneous, ocular, articular, gastrointestinal, and/or central nervous system involvement. - Thrombosis and arterial aneurysms can also occur. • Cogan Syndrome: - Ocular inflammatory lesions-interstitial keratitis, uveitis, and episcleritis as well as inner ear disease Differential Diagnosis: Systemic rheumatic diseases, such as systemic lupus erythematosus, atherosclerotic disease, drug reactions, and vaso-occlusive processes. Among the most important diseases to exclude are infections and malignancies. Presentation - Systemic Symptoms (Sx) in Vasculitis: • Fevers • Fatigue • Anorexia • Weight loss • Night sweats • Arthralgias • Eye inflammation, particularly scleritis • Palpable purpura: strong sign of cutaneous leukocytoclastic vasculitis • Sensory and/or motor neuropathy • Absent, diminished, or tender pulses, bruis, or blood pressure discrepancies Findings by Vessel Size: • Large Vessel: Blood pressure difference in extremities, upper extremity claudication, aortitis, aortic aneurysm • Medium Vessels: Supply gut -> abdominal pain, testes -> pain, infarct • Small Vessels: Skin -> causing palpable purpura. Nerves -> mononeuritis multiplex/neuropathy. Kidneys -> glomerulonephritis. Eye -> retinopathy Lab Tests: • CBC • LFT • ESR • ANA (Suggest CTD) • ANCA (Dx: GPA, EGPA, microscopic polyarteritis, drug-induced vasculitis) - 2 relevant target antigens are proteinase 3 (PR3) and myeloperoxidase (MPO) - (C-ANCA) pattern: antibodies directed against PR3 - (P-ANCA) pattern: is usually directed against MPO • Complement (Low levels mixed cryoglobulinemia, urticarial vasculitis, and SLE) • Urinalysis (Renal involvement) • TSH • SPEP Consider: • Serologies for Cryoglobulins • HIV • HBV HCV • ASO #Vasculitis #Vasculitides #differential #diagnosis #rheumatology #classification
