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.
Service de Rhumatologie, Hôpital d'Adultes de Brabois, CHU de Nancy, 54511 Vandoeuvre-lès-Nancy, France.
Félicie
F
Costantino
Damien
D
Loeuille
Hervé
H
Dintinger
Patrice
P
Péré
Isabelle
I
Chary-Valckenaere
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