ABSTRACT
PURPOSE: Polymyalgia rheumatica (PMR) is a frequent cause for long-term corticosteroid therapy. Management of PMR is difficult and recommendations (regarding diagnosis and treatment) from the British Society of Rheumatology have been recently published in order to avoid false diagnosis and unnecessary corticosteroid therapy. On the other hand, late onset spondyloarthropathies are difficult to diagnose due to their various presentation (peripheral and axial manifestations, usually associated with severe systemic manifestations) and the absence of validated diagnosis criteria in the elderly. METHODS: We report on eight patients, who all of them initially responding to Bird's criteria for PMR, and whose outcome was refractory PMR with multiple flares, poor therapeutic response, with inability to taper steroids. RESULTS: After a mean follow-up of 25 months, a diagnosis of late onset spondyloarthropathy was done in all theses patients based on clinical history, physical examination, and spine MRI. In four of the cases the use of TNFα blockers allowed to taper corticosteroid and to control the disease. Retrospectively, the diagnosis at presentation was difficult. CONCLUSION: Among PMR patients with poor response to corticosteroids and multiple flares, the possibility of a late onset spondyloarthropathy should be discussed. There is an unmet need for validated diagnosis criteria in such patients.
Subject(s)
Diagnostic Errors , Polymyalgia Rheumatica/diagnosis , Spondylarthropathies/diagnosis , Age of Onset , Aged , Diagnosis, Differential , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Spondylarthropathies/epidemiologyABSTRACT
OBJECTIVE: To determine the incidence of renal AA amyloidosis and its association with rheumatoid arthritis (RA) in a cohort of all renal biopsies at one referral hospital and to measure the effect of a monthly pulse of cyclophosphamide on renal function and survival in these RA patients. METHOD: All renal biopsies with proven AA amyloidosis from a single pathology unit linked to a major nephrology referral unit in a university hospital were selected retrospectively and RA patients were identified. We studied 6931 renal biopsies. The effect of treatment with and without pulse cyclophosphamide on renal function and survival was studied in these patients. RESULTS: From March 1977 to February 1999, the incidence of AA amyloidosis was 2.4 cases/yr. The incidence and prevalence of the association of AA amyloidosis with RA were 0.68 cases/yr and 0.22% (15/6931) respectively. RA patients treated with cyclophosphamide (n=6) had a lower rate of renal function loss (P=0.013) and a higher median survival (P=0.026) than untreated patients (n=9). During the follow-up period, two out of six treated patients (33%) and all nine untreated patients (100%) died. CONCLUSIONS: AA amyloidosis is a rare complication of RA and complicates the evaluation of treatment. This retrospective study suggests that treatment with cyclophosphamide is able to reduce the incidence of end-stage renal failure and to increase survival. Prospective studies are needed to clarify this issue.