Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up.

INTRODUCTION: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. PATIENTS AND METHODS: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. RESULT: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72.7%), lower respiratory tract (81.8%) and kidneys (72.7%). URT involvement indicated good prognosis (p= 0.046). Survival in the patients with and without URT involvement was 124.6 ± 6.9 months and 59.7 ± 22.9 months, respectively. End-stage renal failure (ESRF) requiring dilaysis and cardiac involvement were associated with mortality (p= 0.022 and p= 0.026, respectively). Of the 12 dialysis-dependent patients at diagnosis, 11 survived > 3 months and seven regained renal function permanently. Dialysis dependency was significantly lower in patients who received plasmapheresis (p= 0.047). Overall mortality rate was 18% (4/22). Mean survival was 55.9 ± 42.8 months (IR= 84.0). CONCLUSIONS: Diagnosis of GPA may be delayed by the nonspecific nature of its symptoms. URT involvement was associated with good prognosis, whereas cardiac involvement and ESRF requiring dialysis were associated with poor outcome. Plasmapheresis may increase the rate of renal recovery in the patients with ESRF requiring dialysis.

Serum Immunoglobulin G4 levels are elevated in patients with Graves' ophthalmopathy.

OBJECTIVE: Recent studies have shown close association between serum Immunoglobulin G4 (IgG4) levels and forms of autoimmune thyroiditis. However, there are limited data about the relationship between IgG4 and Graves' ophthalmopathy (GO). In the present study, we aimed to determine the possible association between IgG4 and GO. DESIGN: Cross-sectional study. PATIENTS: Sixty-five patients with Graves' disease (GD) and 25 healthy controls were recruited into the study. Thirty-two of these patients had GO. MEASUREMENTS: Serum IgG4 levels, thyroid functions and thyroid volumes were measured in all participants. Ophthalmological examination including Hertel's exophthalmometer readings (HER), Schirmer's test (ST), 'NO SPECS' classification and clinical activity score evaluation (CAS) were performed to all patients with GD. RESULTS: IgG4 levels were significantly elevated in patients with Graves' disease compared to controls (P = 0·0001). Also, IgG4 levels were significantly higher in patients with and without GO when compared to control subjects (P = 0·0001 and P = 0·002, respectively). Furthermore, IgG4 levels were significantly higher in the GO group compared with GD patients without GO (P = 0·024). IgG4 levels were observed to increase in parallel to CAS. Compared with other GD patients, 15 GD patients with serum IgG4 levels ≥ 135 mg/dl had higher CAS scores (P = 0·012). None of the factors including, TSH, T3, T4 levels, thyroid volume, HER and ST measurements, affect IgG4 levels as an independent factor. CONCLUSION: IgG4 levels are evidently increased in patients with GD, and there is a possible relationship between IgG4 and GO. Our results suggest that IgG4 may be helpful in screening GD patients with high risk for GO and may well become a good indicator for the selection of right medication in the future.

Effects of infliximab treatment in terms of cardiovascular risk and insulin resistance in ankylosing spondylitis patients.

OBJECTIVE: To assess the effects of infliximab treatment on insulin sensitivity and cardiovascular risk factors in patients with ankylosing spondylitis (AS). METHODS: In this prospective study, 30 consecutive AS patients (23 men and 7 women) fulfilling the modified 1984 New York criteria for AS were investigated. All patients were treated with intravenous infliximab. A complete biochemical profile and assesments were obtained before and after 12 weeks of infliximab therapy. The Homoeostasis Model Assessment of Insulin Resistance Index (HOMA-IR) was used to measure insulin resistance (IR). Framingham equation was used to assess cardiovascular risk factors. RESULTS: After 12 weeks of infliximab treatment, there was no statistically significant difference in fasting insulin, HOMA-IR, lipid parameters, body-mass index, waist circumference and waist-hip ratio, whereas fasting glucose levels (p = 0.001), triglycerides/high-density lipoprotein (HDL) ratio (p = 0.043) and total cholesterol/HDL (p = 0.041) ratio increased significantly from baseline. A significant decrease was observed for both systolic blood pressures (p < 0.001) and diastolic blood pressures (p = 0.003) in the 12th-week visit. A significant decrease was also found in terms of Framingham risk scores (p = 0.028) after treatment. CONCLUSIONS: Study results suggest that infliximab treatment may reduce cardiovascular risk and blood pressures without changing IR.

If Neurologists Establish The Diagnosis of Primary Sjogren's Syndrome?

INTRODUCTION: Neurological involvements were shown in 20% of patients with Primary Sjogren's Syndrome (pSS). Neurological symptoms may be the first signs of pSS in 57% of the cases. In addition, early diagnosis and treatment of neurological disorders may save or improve the quality of life of these cases. There have been reports about the neurologic manifestations of pSS but little is known about the details of neurologically presented cases. METHOD: In this study, we described 11 pSS patients who presented with neurological manifestations. RESULTS: Central nervous system (CNS) involvement was recorded in 7 (63.7%) and peripheric nervous system (PNS) involvement in 4 cases (36.4%). CONCLUSION: Our findings regarding the cases with neurological manifestations leading to the diagnosis of pSS suggest that: 1) The frequency of CNS involvement was higher than that of PNS, and the most frequent clinical pictures of CNS involvement are Multiple Sclerosis (MS)-like illnesses and optic neuritis, 2) Guillain Barre Syndrome (GBS) was the most frequent disease of PNS involvement; 3) Mononeuropathy multiplex (MM) might be the first sign of pSS; 4) Neurologists should consider pSS in the differential diagnosis of cases with MS, optic neuritis, GBS and neuropathies of unknown causes including MM; 5) There is an urgent need of therapeutical guidelines for the cases with neurological involvement associated with pSS.

Correction of hypothyroidism seems to have no effect on body fat.

Aim. We aimed to observe the effects of L-thyroxine replacement therapy on body fat content determined with various anthropometric methods and a bioelectrical impedance analysis method in patients with hypothyroidism. Methods. Forty-two women with naive autoimmune hypothyroidism were included. Also, 40 healthy participants were enrolled as a control group. Weight, body mass index, waist circumference, and subscapulary, suprailiac, femur, biceps, and triceps skin fold thicknesses were measured. Body fat percentages were calculated and body fat measurements were performed. Euthyroidism was maintained with L-thyroxine. At the 6th and 18th month, of therapy, measurements were reperformed. Results. Mean TSH levels were 57.49 ± 36.46 mIU/L in hypothyroid group and 1.94 ± 1.12 mIU/L in control subjects at admission. In hypothyroid patients, calculated body fat percentages were greater than those of the control subjects during follow-up. Body fat percentage of each hypothyroid case decreased at 6- and 18-month controls, but the decrements were statistically insignificant. Although skin fold thicknesses measured from all sites were observed to decline, only those obtained from femur and biceps showed a significant decrease (P = 0.03 and P = 0.01, resp.). Discussion. Correction of hypothyroidism did not cause any improvement in body weight and body fat percentage. The decrease in skin fold thicknesses might probably result from the reduction in subcutaneous mucopolysaccharide deposits.

Successful management of a giant pituitary lactosomatotroph adenoma only with cabergoline.

Although advances in endocrinologic and neuroradiologic research allow easier recognition of pituitary adenomas, giant pituitary tumours are relatively rare. In the literature, the term "giant" is generally used when a pituitary tumour becomes larger than 4 cm in diameter. Cabergoline is a potent and long-acting inhibitor of prolactin secretion, which exhibits high specificity and affinity for dopamine D2 receptor. Herein, we report a 46-year-old woman with a giant lactosomatotroph pituitary adenoma, sized 6 × 5 × 5.5 cm, who is treated successfully only with cabergoline. The patient showed dramatic response to cabergoline treatment by means of clinical, biochemical and radiological imaging findings. Cabergoline seems to be safe and effective in the treatment of prolactin and growth hormone cosecreting pituitary adenomas as well as prolactinomas. However, surgical or more aggressive approach must be considered where indicated.