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ABSTRACT: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by recurrent self-limited attacks
of fever, accompanied with peritonitis, pleuritis or arthritis. It is well known that FMF may coexist with vasculitic pathologies,
especially with those involving small and medium vessels. Among the vasculitic pathologies reported to be associated with
FMF, Henoch–Schönlein purpura and polyarteritis nodosa come the first, possibly followed up by protracted febrile myalgia.
However, coexistence of FMF with any large vessel vasculitis has not been reported to date. Here, we present a case with FMF
who later developed Takayasu arteritis, with a severe disease course, being resistant to corticosteroids and conventional
immunosuppressive agents, and requiring infliximab treatment.
KeywordsFamilial Mediterranean fever–Takayasu’s arteritis
Rheumatology International 04/2012; · 1.88 Impact Factor
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ABSTRACT: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by recurrent self-limited attacks of fever, accompanied with peritonitis, pleuritis or arthritis. It is well known that FMF may coexist with vasculitic pathologies, especially with those involving small and medium vessels. Among the vasculitic pathologies reported to be associated with FMF, Henoch-Schönlein purpura and polyarteritis nodosa come the first, possibly followed up by protracted febrile myalgia. However, coexistence of FMF with any large vessel vasculitis has not been reported to date. Here, we present a case with FMF who later developed Takayasu arteritis, with a severe disease course, being resistant to corticosteroids and conventional immunosuppressive agents, and requiring infliximab treatment.
Rheumatology International 03/2011; 32(6):1675-8. · 1.88 Impact Factor
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ABSTRACT: Smoking is well known to contribute to the pathogenesis and severity of some systemic autoimmune rheumatic diseases and especially to the production of certain autoantibodies. Primary Sjögren's syndrome (pSS) is an autoimmune disease, affecting primarily the exocrine glands. It may also cause extraglandular involvement in some cases. In this study, we aimed to determine the frequency of smoking habits in our cohort of pSS patients and to investigate whether the frequencies of autoantibody positivity and extraglandular involvement were significantly different between patients with and without smoking. In this cross-sectional study, 207 patients with pSS (F/M 203/4), fulfilling the United States-European Consensus Criteria, and 602 healthy controls (F/M 534/68) were included. Patients and controls were classified into five groups: never smokers, current smokers, former smokers; ever smokers, and passive smokers. The χ(2) and Kruskal-Wallis tests were used for statistical analysis; a p value of less than 0.05 was accepted as statistically significant. While the frequency of current smokers was significantly lower in the pSS group compared with the healthy controls (11.6 vs 22.3%), the frequencies of former smokers (30.4 vs 11.8%), ever smokers (42.0 vs 34.1%), and passive smokers (47.3 vs 37.5%) were significantly higher in the pSS group compared with the healthy controls. In pSS patients, only antinuclear antibody (ANA) positivity was significantly associated with smoking habits, while there was no significant association with other autoantibodies or with the presence of extraglandular involvement. We found that in pSS patients smoking was significantly associated only with ANA positivity. Unlike the deleterious effects of smoking upon disease severity and anti-cyclic citrullinated protein (CCP) antibody production in rheumatoid arthritis, we could not find any association of smoking with extraglandular involvement and/or anti-Ro/anti-La antibody positivity in pSS. These results are indeed in line with the limited number of previous studies reported in the literature. Further studies with higher numbers of pSS patients are required to confirm the seemingly negative association of smoking with pSS.
Modern Rheumatology 03/2011; 21(6):602-7. · 1.58 Impact Factor
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ABSTRACT: Chronic interstitial cystitis (IC), mostly affecting middle-aged women, is a very rare manifestation of primary Sjögren's syndrome (pSS). Hereby, we report a 42-year-old woman with pSS, presenting with dysuria, urinary frequency, and suprapubic pain. She was diagnosed to have chronic IC, based upon the cystoscopic biopsy finding of chronic inflammation in the bladder wall. Systemic corticosteroid and azathioprine treatments together with local intravesical therapies were not effective. Therefore, cyclosporine (CSA) therapy was initiated. Initial low dose of CSA (1.5 mg/kg/d) improved the symptoms of the patient, with no requirement for dose increment. After 4 months of therapy, control cystoscopic biopsy showed that bladder inflammation regressed and IC improved. This case suggests that even low doses of CSA may be beneficial for treating chronic IC associated with pSS syndrome.
Rheumatology International 01/2011; 32(5):1215-8. · 1.88 Impact Factor
