Peritoneal amyloidosis with myopathy in primary systemic (AL) amyloidosis

Description

We present an unusual case of primary systemic amyloidosis presenting with myopathy and peritoneal amyloid deposition. Peritoneal amyloid is a rare disease with few published cases.1

An 85-year-old man was referred to rheumatology with walking difficulties and elevation of serum creatine kinase (CK). Initial tests revealed elevated erythrocyte sedimentation rate (ESR) 76 mm/hour; normal C reactive protein (CRP) and serum CK of 525 IU/L. Antinuclear and extractable nuclear antibodies (including anti-Jo1) were negative. Investigations for myopathy included MRI Stir sequence (figure 1) demonstrated increased signal in the hip adductors bilaterally, consistent with symmetrical myositis. CT scan of the chest, abdomen and pelvis, requested to exclude occult cancer, revealed diffuse infiltration of the mesentery and retroperitoneal fat (figure 2), despite lack of abdominal symptoms. This appearance was initially considered suspicious of malignancy without an obvious primary lesion; however, mesenteric biopsy reported only hyaline fatty...

http://ift.tt/2kAnXzs