RESUMO
OBJECTIVES: We aimed to investigate serum cystatin C (cysC) levels in primary Sjögren's syndrome (pSS) patients, and evaluate its correlation with renal involment. MATERIALS AND METHODS: Eighty-six pSS patients and 65 age- and gender-matched healthy controls were enrolled into the study. Serum cysC, urea, serum creatinine (SCr), creatinine clearance (CrCl), glomerular filtration rates (GFR), Na, K, Mg, Ca, uric acid, P, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), anti-Ro/SS-A, anti-La/SS-B, antinuclear antibodies, 24-h urinary poteinuria and microalbuminuria were evaluated. RESULTS: Mean serum cysC levels did not differ between the patients and healthy controls (P > 0.05). Nine patients with pSS had proteinuria over 150 mg (and microalbuminuria over 30 mg) per 24 h. In patients with proteinuria, serum cysC levels correlated with serum K (r = 0.279, P = 0.024), ESR (r = 0.405, P = 0.001) and the disease duration (r = 0.235, P = 0.04), respectively. Patients with positive anti-Ro/SS-A and anti-La/SS-B antibodies had higher SCr levels compared to those with negative serology (r = 0.292, P = 0.009, and r = 0.259, P = 0.022, respectively). Nine patients with proteinuria and anti-Ro/SS-A, anti-La/SS-B positivity tended to have lower K and Mg levels which suggests subclinical renal tubular acidosis. CONCLUSION: There were no associations between serum cysC levels and renal involvement in patients with pSS. However, in patients with proteinuria, serum cysC levels were correlated with acute-phase reactants, suggesting an association with disease activity in terms of degree of inflammation.
Assuntos
Cistatina C/análise , Nefropatias/sangue , Síndrome de Sjogren/sangue , Adulto , Albuminúria/sangue , Albuminúria/etiologia , Biomarcadores/sangue , Sedimentação Sanguínea , Estudos de Casos e Controles , Feminino , Taxa de Filtração Glomerular , Humanos , Rim/fisiopatologia , Nefropatias/diagnóstico , Nefropatias/etiologia , Nefropatias/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnósticoAssuntos
Artrite Reumatoide/etiologia , Febre Familiar do Mediterrâneo/complicações , Sarcoidose/etiologia , Adulto , Anti-Inflamatórios/uso terapêutico , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/imunologia , Autoimunidade , Quimioterapia Combinada , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/tratamento farmacológico , Febre Familiar do Mediterrâneo/imunologia , Feminino , Humanos , Imunossupressores/uso terapêutico , Pessoa de Meia-Idade , Sarcoidose/diagnóstico , Sarcoidose/tratamento farmacológico , Sarcoidose/imunologia , Resultado do TratamentoRESUMO
Familial Mediterranean fever (FMF) is an inherited disease characterized by recurrent episodes of fever and serositis. FMF is caused by mutations in the MEFV gene that encodes pyrin/marenostrin. The 5 most frequent mutations are M694V, M694I, V726A, M680I and E148Q. Here, we reported 3 FMF patients, a sister and two brothers, who have the same M694V mutation with different clinical presentations. While the sister presented with abdominal pain, one of the brothers presented with erysipelas-like erythema and the other brother with bilateral sacroiliitis. Here, we report the different clinical courses of FMF in a family carrying the same M694V mutation.
