BOARD BASICS: vasculitis

older adults with fever, headaches, scalp tenderness, jaw claduciation, and visula symptoms

Giat cell arteritis

commonly associated with giant cell arteritis or developds as a primary condition

polymyalgia rheumatica

older adults with aching and morning stiffness in the proximal muscles of the shoulder and hip girdle

muscle strength and muscle enzymes are normal

ESR usually >50

polymyalgia rheumatica

young women with fever, malaise, weight loss, and arthralgia preceding arm/leg claudication, pulse deficits, vascular bruits, and asymmetric arm BP readinings

Takayasu arteritis

nonglomerular kidney disease, hypertension, mononeuritis multiplex, and skin lesions (nodules, livedo reticularis, palpable purpura)

polyarteritis nodosa

recurrent headaches, stroke, TIA, and progressive encephalopathy

primary angiitis of the CNS

pulmonary infiltrates, palpable purpura, and rapidly progressive pauci-immune GN

microscopic polyangiitis

also called leukocytoclastic vasculitis

hypersensitivity vasculitis

palpable purpura (lower legs), cutaneous vesicles, pustules, maculopapular lesions, urticaria, recent viral infection, drug exposure, or diagnosis of malignancy

hypersensitivity vasculitis

skin lesions (red macule, palpable purpura, nodules, or ulcers) GN, mononeuritis multiplex, and elevated serum aminotransferase levels

cryoglobulinemic vasculitis

treatment: initial high dose glucocorticoids plus tocilizumab; treat immediately to prevent blindness and obtain biopsy in <2 weeks

Giatn cell arteritis

treatment: low dose prednisone; relapse common and prolonged courses typical (1-3 years)

polymyalgia rheumatica

treatment: prednisone, steroid-sparing agents such as methotrexate or azathioprine

Takayasu arteritis

treatment: glucocorticoid and cyclophosphamide for severe organ threatening disease; treat concomitant HBV infection

polyarteritis nodosa

treatment: prednisone and cyclophosphamide

primary angiitis of the CNS

treatment: prednisone and either rituximab (preferred) or cyclophosphamide

GPA and MPA

treatment: prednisone; cyclophosphamide or mepolizumab is added for severe multiorgan disease

EGPA

treatment: typically self limited; glucocorticoids or cyclophosphamide for severe, persistent GN

IgA vasculitis

treatment: treat underlying HCV infix. if organ dysfunction is severe, also treat with prednisone, cyclophosphamide, and plasmapheresis

cryoglobulinemic vasculitis

treatment: prednisone; steroid sparing agents may be required for major disease manifestations (uveitis, CNS, GI, or large artery involvement); colchicine or apremilast for oral ulcers

Behcet syndrome