Fixed digital contractures revealing light-chain amyloidosis.
Among the many clinical manifestations of light-chain (AL) amyloidosis, musculoskeletal involvement is rarely reported. We describe the case of a 72-year-old woman who was referred to our rheumatology department for fixed flexion contractures of the fingers that developed concomitantly with a decline in general health. Macroglossia and recent-onset dyspnea were noted. Investigations, which included a tongue biopsy, established the diagnosis of kappa light-chain amyloidosis with soft-tissue, bone and cardiac deposits. Melphalan and dexamethasone therapy was successful in stabilizing the clinical and laboratory abnormalities within 6 months. This case is remarkable in that the musculoskeletal manifestations were at the forefront of the clinical picture and led to the diagnosis.
Costantino
Félicie
F
Service de Rhumatologie, Hôpital d'Adultes de Brabois, CHU de Nancy, 54511 Vandoeuvre-lès-Nancy, France.
Loeuille
Damien
D
Dintinger
Hervé
H
Péré
Patrice
P
Chary-Valckenaere
Isabelle
I
D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis* / Q000276:immunology*; D003286:Contracture / Q000000981:diagnostic imaging / Q000209:etiology*; D004417:Dyspnea / Q000175:diagnosis; D005260:Female; D019788:Fluorodeoxyglucose F18; D050276:Hand Bones / Q000000981:diagnostic imaging; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology; D007145:Immunoglobulin kappa-Chains / Q000276:immunology; D008260:Macroglossia / Q000209:etiology*; D011859:Radiography; D011868:Radioisotopes; D012785:Shoulder Joint / Q000000981:diagnostic imaging
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D011868:Radioisotopes; D019788:Fluorodeoxyglucose F18