title: Inflammatory myopathy as the initial presentation of cryoglobulinaemic vasculitis.
abstract: Cryoglobulinaemic vasculitis is characterised by immunoglobulin deposition at low temperatures. The most common manifestations are cutaneous involvement, arthralgias, Raynaud's phenomenon, peripheral neuropathy and renal disease. Myopathy is unusual and only a few cases have been reported. Here, we present a 31-year-old woman who developed progressive muscle weakness involving upper and lower extremities, dysphagia, paraesthesias and palpable purpura. Diagnostic studies revealed elevated creatine kinase, diffuse myopathic and sensorimotor axonal neuropathy on electromyography and nerve conduction studies, and inflammatory myopathy on muscle biospsy. Cryoglobulin levels were elevated on two occasions. She responded favourably to cyclophosphamide and high-dose corticosteroids. Cyclophosphamide was continued for 1 year followed by methotrexate. Prednisone was gradually tapered and discontinued 1 year later. She remained in clinical remission after 4 years of follow-up. This case suggests that cryoglobulinaemia should be considered in the differential diagnosis of a patient presenting with inflammatory myopathy.
authors.affiliation: Department of Medicine, Division of Rheumatology, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico.
authors.forename: Noelia
authors.initials: N
authors.lastname: Rodríguez-Pérez
authors.forename: Yerania
authors.initials: Y
authors.lastname: Rodríguez-Navedo
authors.forename: Yvonne M
authors.initials: YM
authors.lastname: Font
authors.forename: Luis M
authors.initials: LM
authors.lastname: Vilá
mesh_terms: D000328:Adult; D003449:Cryoglobulinemia / Q000175:diagnosis* / Q000188:drug therapy; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008775:Methylprednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D009220:Myositis / Q000175:diagnosis* / Q000188:drug therapy; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D016896:Treatment Outcome; D014657:Vasculitis / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D003520:Cyclophosphamide; D011241:Prednisone; D008775:Methylprednisolone