Systemic amyloidosis AL with temporal artery involvement revealing lymphoplasmacytic malignancy in a man presenting as polymyalgia rheumatica.
A 68 year old man presented with a clinical and biological picture that suggested polymyalgia rheumatica. Temporal artery biopsy disclosed no inflammatory change but massive light chain amyloid deposits in the media. Further exploration showed a malignant lymphoplasmacytic haemopathy with a triclonal gammopathy and a muscular, rectal, and probable cardiac amyloidosis. Cryoglobulinaemia and high concentrations of soluble interleukin 2 receptor (sIL-2R) were also found. This is the fifth case with confirmed involvement of the temporal artery. The especially high sIL-2R concentration was thought to reflect the tumour mass rather than lymphocyte activation.
Lafforgue
P
P
Department of Rheumatology, Timone Hospital, Marseilles, France.
Senbel
E
E
Figarella-Branger
D
D
Boucraut
J
J
Horschowsky
N
N
Pellissier
J F
JF
Acquaviva
P C
PC
D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D006402:Hematologic Diseases / Q000150:complications*; D006801:Humans; D008297:Male; D009135:Muscular Diseases / Q000473:pathology; D011111:Polymyalgia Rheumatica / Q000175:diagnosis*; D013699:Temporal Arteries