Multiple bone fracture due to Fanconi\u27s syndrome in primary Sjogren\u27s syndrome complicated with organizing pneumonia.

A 66-year-old woman showing renal dysfunction with elevated serum alkaline phosphatase and anti-SS-A antibody was admitted. A labial salivary gland biopsy showing infiltration of mononuclear cells and positive anti-SS-A antibody with sicca symptoms led to a diagnosis of primary Sjogren\u27s syndrome (SS). Fanconi\u27s syndrome was diagnosed by renal tubular acidosis along with renal glucosuria or aminoaciduria and multiple bone fractures on bone scintigraphy. Typical bilateral pulmonary shadows were confirmed as organizing pneumonia (OP) determined by the analysis of bronchoalveolar lavage fluid and transbronchial lung biopsy. A rare complication of Fanconi\u27s syndrome with OP in SS is described

The liver is a common non-exocrine target in primary Sjögren's syndrome: A retrospective review

BACKGROUND: The autoimmune destruction of exocrine glands that defines primary Sjögren's syndrome (1°SS) often extends to non-exocrine organs including the liver. We aimed to determine the prevalence of liver disease in patients with 1°SS and to evaluate the association of this complication with other non-exocrine features and serologic markers of autoimmunity and systemic inflammation. METHODS: We reviewed 115 charts of patients with 1°SS and further analyzed the 73 cases that fulfilled the European Epidemiology Center Criteria, seeking evidence for clinical and subclinical liver disease. RESULTS: Liver function tests had been determined in 59 of the 73 patients. Of those, 29 patients (49.1%) had abnormal liver function tests including 20.3% with clinically overt hepatic disease. Liver disease was the most common non-exocrine feature in this cohort. Risk factors for abnormal liver function tests were distributed similarly between the patients with and without liver disease. In 60% of patients with abnormal liver function tests no explanation for this complication was found except for 1°SS. Liver involvement was significantly more common in 1°SS patients who also had evidence of lung, kidney and hematological abnormalities. Patients with abnormal liver function tests were also more likely to have an elevated sedimentation rate and a positive anti-ENA during the course of their disease. CONCLUSION: Liver involvement is a common complication in 1°SS. Its presence correlates with systemic disease. We consider that this complication should be routinely sought in patients with 1°SS, especially when a positive anti-ENA or evidence of systemic inflammation is found

LIPID ABNORMALITIES IN CHRONIC UREMIC PATIENTS - RESPONSE TO TREATMENT WITH GEMFIBROZIL

Seventy-four patients with end stage renal failure were studied.Forty-six of them were on hemodialysis (HD) while 28 were on continuous ambulatory peritoneal dialysis (CAPD). In addition 56 non-dialysis chronic renal failure (NDCRF) patients with various degree of renal failure were also studied. In all groups serum triglyceride concentrations were significantly higher and HDL cholesterol concentrations were significantly lower compared to age- and sex-matched controls. Total and LDL cholesterol were significantly higher in the NDCRF and CAPD patients compared to controls. In 55 patients (20 on HD, 13 on CAPD and 22 NDCRF) with severe hypertiglyceridemia or diminished HDL cholesterol gemfibrozil 300 mg b. i. d per os was given for 6 months. Drug treatment reduced significantly serum triglycerides in all groups of patients and increased the levels of HDL cholesterol in CAPD patients. Moreover, a statistically significant decrease of the levels of total and LDL cholesterol was noticed in HD and NDCRF patients. During treatment no significant side effects were observed and liver and muscle enzymes remained within normal values