Amyloidosis secondary to rheumatic diseases - 16 cases.

INTRODUCTION: Secondary amyloidosis (SA) results of tissue deposition of an acute phase reactant protein produced by chronic inflammation. Its incidence appears to be declining, following the improvement of medical care to primary diseases. Our aim is to assess a group of Portuguese patients with amyloidosis secondary to inflammatory rheumatic diseases, and their evolution over the past 10 years. METHODS: The study comprised 16 patients with SA confirmed by tissue biopsy, hospitalized in the Rheumatology Department of Hospital São João in Oporto in the last 10 years. We made a protocol on epidemiological, clinical and analytical data focusing the rheumatic disease and SA, and possible elements of connection between them. RESULTS: Of the 16 patients, mainly women (81,2%), with mean age at entry of 56 years, 68,8% had rheumatoid arthritis. Amyloidosis was diagnosed in average at 13,5 years of primary rheumatic disease, and its main manifestation was kidney involvement, which together with infection and orthopaedic surgery or its complications, were the leading causes of hospitalization. In this time interval, 6 patients died. They were older, with longer duration and lower rate of treatment of the primary rheumatic disease, and had SA diagnosed 1,5 years before death (different of the 5 years of those that still alive). They had higher rate of gastrointestinal, neurological and serious kidney involvement, and hospitalizations. CONCLUSIONS: Improving medical care in rheumatic inflammatory diseases has reduced the incidence of SA. Also, biotherapy appears to be achieving positive results in established amyloidosis, whatever the mechanisms involved. Our data, on Portuguese patients, seems to follow this trend.

[Systemic lupus erythematosus: imaging of central nervous system involvement by magnetic resonance]. / Lupus eritematoso sistémico: a ressonância magnética no envolvimento do sistema nervoso central.

UNLABELLED: Patients with systemic lupus erythematosus (SLE) can develop dysfunction of the central nervous system (CNS) and have several neurologic syndromes with heterogeneous pathogenic mechanisms. The most common neurologic manifestations of SLE are organic mental syndromes, functional psychoses and seizures. METHODS: We retrospectively reviewed the magnetic resonance imaging (MRI) of SLE patients with CNS involvement followed in S. João Hospital Rheumatology clinic. Only patients with abnormal MRI were selected. RESULTS: In 42 patients studied there were 22 (52,4%) cases with abnormal MRI. Patient's age ranged between 20 and 63 years (mean 35,6). All were females. MRI showed multiple periventricular white matter lesions in 16 cases (68,2%) and cerebral infarctions in 3 cases (13,6%). There were atrophic changes in 4 cases (18,2%) and cerebral hemorrhage in 2 cases (9,1%). There was a case (4,5%) of transverse myelitis associated with acute optic neuritis (Devic's syndrome) and 1 case (4,5%) of predominantly posterior white matter signal abnormalities (reversible posterior leukoencephalopathy syndrome). CONCLUSION: This study confirms MRI importance on in identifying and characterizing CNS abnormalities, which contributes to better investigation and management of these patients. The authors discuss etiological possibilities of the different imagiological findings.