Cryoglobulinemic vasculitis

(Lancet 2012;379:348; Nat Rev Dis Primers 2018;4:11)

• Cryoglobulins: proteins that precipitate from serum or plasma on exposure to cold and redissolve on rewarming, characterized by their composition; a/w chronic immune stimulation and/or lymphoproliferation
• Distinguish from cryofibrinogenemia = proteins (eg, fibrin, fibrinogen) that precipitate only from plasma; found in autoimmune dis, malignancies, infxns; unclear clinical significance

Types of Cryoglobulinemia (J Autoimmun 2019;105:102313)

• Epidemiology: ~1/100,000, but prevalence varies with HCV rates; ♀ >♂
• Etiologies (idiopathic in ~10%) Hematologic diseases: multiple myeloma, MGUS, Waldenström’s, chronic lymphocytic leukemia in type I; B-cell lymphomas or solid-organ malignancies in type II Infxns (types II & III): viral (HCV [>80% RNA ⊕], HBV, HIV, HAV, EBV, CMV), bacterial (endocarditis, strep, etc.), fungal (coccidiomycosis, etc.), parasitic (malaria, amoebiasis) Autoimmune syndromes (type III >II): Sjögren’s syndrome, SLE, RA, PAN Renal transplant recipients (Clin Nephrol 2008;69:239)
• Pathophysiology of Cryoglobulinemic vasculitis
• Clinical manifestations of Cryoglobulinemic vasculitis (most Pts w/o sx)
• Dx studies of Cryoglobulinemic vasculitis
• Treatment of Cryoglobulinemic vasculitis