AB1306 AA AMYLOIDOSIS IN CHRONIC RHEUMATIC DISEASES: ABOUT 20 CASES

Abstract

BackgroundSecondary AA amyloidosis is a complication of certain uncontrolled chronic inflammatory rheumatic diseases. The diagnosis of AA amyloidosis is anatomo-pathological based on the detection of amyloid deposits within a biopsy specimen.ObjectivesThe aim of this study is to determine the clinical, biological, therapeutic and evolutionary aspects of amyloidosis during these diseases.MethodsThis is a retrospective descriptive study conducted in the rheumatology department in Monastir, Tunisia, including patients followed for chronic inflammatory rheumatism complicated by AA amyloidosis.ResultsOur study included 20 patients (13 women and 7 men). Their mean age at the time of diagnosis of amyloidosis was 55.4 years [18-76]. The rheumatic diseases were: Rheumatoid Arthritis (RA) in 13 cases (65%), Ankylosing Spondylitis in 3 cases (15 %), Behçet’s disease in 2 cases and Juvenile idiopathic arthritis in its systemic form in 2 cases (10% for both). Amyloidosis occurred after a mean duration of rheumatic disease of 11.5 years [3-27]. The mean sedimentation rate at diagnosis of amyloidosis was 100.5 mm per hour [21-131]. The mean C-reactive protein at diagnosis was 38.35 mg/L [1.4-135]. Inaugural manifestations of amyloidosis were: edema of lower limbs in 8 cases, nephrotic syndrome in 7 cases, renal failure in 4 cases and an abdominal clinical presentation (diarrhea and abdominal pain) associated with proteinuria in 2 cases. Proteinuria was constant, with a mean level of 7.5 g/24 h [3.5-20]. It was isolated in 5 cases. Amyloidosis was subclinical in 2 cases and was discovered by an abdominal fat biopsy performed during a research protocol. The diagnosis was confirmed by: renal biopsy (n=9), rectal biopsy (n=5), salivary gland biopsy (n=4) and abdominal fat biopsy (n=2). Amyloidosis extension was assessed for 50% of our patients. Cardiac involvement was the most frequent, revealed in 5 patients. Concentric left ventricular hypertrophy was found in 4 patients. Pulmonary arterial hypertension and pericardial effusion were found in 1 case. Hepatic involvement was detected in 2 cases in the form of hepatomegaly without disturbance of liver function tests. Adrenal gland involvement was detected in 1 case with homogeneous hypertrophy of the adrenal glands associated with adrenal dysfunction. Only 1 patient had peripheral nerve damage in the form of bilateral carpal tunnel syndrome. Concerning the treatment; 12 patients received Colchicine. Chloraminophen was tried for 4 patients with amyloidosis secondary to RA and Azathioprine for one patient with RA. The evolution was unfavorable in 6 cases marked by death within a mean time of 3.4 years.ConclusionAlthough its prevalence is decreasing, owing to therapeutic progress in chronic rheumatic diseases, AA amyloidosis remains a not exceptional complication. It has a poor prognosis, particularly because of renal disease and cardiac involvement.Disclosure of InterestsNone declared