AL amyloidosis presenting as inflammatory polyarthritis: a case report.

Amyloidosis is a condition characterised by extracellular tissue deposition of fibrils causing a wide range of clinical manifestations. This protein deposition can occur in any tissue, most commonly in the kidney, heart, skin, peripheral nervous system, and gastrointestinal tract. However, the deposition of amyloid fibrils in the synovium is seldom reported. Musculoskeletal manifestations are subtle, subclinical and rarely the patient presents with symptoms that resemble rheumatic diseases. Here, we describe a 55-year-old man with AL (amyloid light chain) amyloidosis who presented with inflammatory polyarthritis with significant morning stiffness, inflammatory low back pain, and painful thickened tongue. The patient had anaemia, macroglossia with lateral scalloping of the tongue, papules, and plaques in the periocular, perioral and perinasal area, bilateral carpal tunnel syndrome, localised soft tissue swelling over the joints, restricted movement in different joints with flexion contractures in some joints. Rheumatoid factor and ACPA were negative and the X-ray of the sacroiliac joints was normal. We confirmed amyloidosis by biopsy of an affected skin lesion. In the urine, no Bence Jones protein was found and bone marrow study and x-ray of the skull were normal. Plasma immuno-electrophoresis and serum free light chain (FLC) assay confirmed lambda light chain type monoclonal gammopathy. Take home message: Although AL amyloidosis is a rare condition, it should be considered while evaluating atypical symptoms in patients presenting with rheumatic complaints. A high index of suspicion is necessary for proper diagnosis as delay in diagnosis will yield a poorer treatment outcome.

Translation, cross-cultural adaptation and validation of the Pain Catastrophizing Scale (PCS) into Bengali in patients with chronic non-malignant musculoskeletal pain.

AIM: To develop a culturally adapted and validated Bengali Pain Catastrophizing Scale (BePCS). METHODS: The English PCS was translated, adapted and back-translated into and from Bengali, pre-tested by 30 adult patients with chronic non-malignant musculoskeletal pain. The BePCS was administered twice with 14 days interval to 90 patients. Convergent validity was measured by comparing the BePCS score with scores of the domains physical functioning and mental health of the Bengali Short Form 36, through Spearman's correlation coefficient. Test-retest reliability was assessed by intraclass correlation coefficient (ICC) and Spearman's rank correlation coefficient and internal consistency by Cronbach's alpha. Content validity was assessed by index for content validity (ICV) and floor and ceiling effects. RESULTS: The BePCS was well accepted by the patients in the pre-test. The content validity was excellent, both item ICV and scale ICV were 1. Construct validity: the convergent validity was -0.424 for physical functioning and -0.413 for mental health, indicating a moderate negative correlation. Total BePCS score showed excellent internal consistency with a mean Cronbach's α = 0.92. Internal consistency for subscales rumination, magnification and helplessness, were Cronbach's α 0.903, 0.72 and 0.872 respectively. The test-retest reliability of total BePCS was 0.78 (P < .001) and for the subscales rumination 0.872 (P < .001), magnification 797 (P < .001) and helplessness 0.927 (P < .001), showing excellent test-retest reliability. CONCLUSIONS: The interviewer-administered BePCS appears to be an acceptable, reliable and valid instrument for measuring health-related quality of life in Bengali speaking patients with chronic non-malignant musculoskeletal pain. Further evaluation in the general population and in different medical conditions should be done.