Incidence of pemphigoid diseases in Northern Germany in 2016 - first data from the Schleswig-Holstein Registry of Autoimmune Bullous Diseases.

BACKGROUND: Autoimmune bullous diseases (AIBD) are rare disorders characterized by autoantibody formation against components of adhesion molecules; in pemphigoid diseases (PD), these are proteins of hemidesmosomes and basement membrane, important for cell-matrix adhesion in skin and/or mucous membranes. Incidences of these diseases vary considerably between different populations. OBJECTIVES: To establish a registry prospectively recruiting all AIBD patients in a geographically well-defined region in Northern Germany (Schleswig-Holstein). METHODS: Only patients with verified disease (by clinical presentation, histology, direct and/or indirect immunofluorescence and /or ELISA) living in Schleswig-Holstein were included. Incidences of PD were estimated based on the total number of inhabitants in Schleswig-Holstein, stratified by birth year and sex. RESULTS: Of 67 patients with PD [35 male, 32 female, mean age 75 (standard deviation 14.3 years)], 83% were patients with bullous pemphigoid [n = 56, 28 male, 28 female, mean age 78 (SD 9.9)]. The resulting crude incidences were 23.4 patients/million/year for all pemphigoid patients, 19.6 patients/million/year for bullous pemphigoid (age-standardized 16.9 patients/million/year) with a strong increase in bullous pemphigoid patients in the age group of 85-90 years with 262 patients/million/year. Incidences for bullous pemphigoid were higher in urban compared to rural areas. Other PD (mucous membrane pemphigoid, linear IgA disease, anti-p200 pemphigoid) were less frequent with crude incidences of 2.1, 1.0 and 0.7 patients/million/year, respectively. CONCLUSIONS: This study prospectively analyses the incidence of PD in a carefully defined geographical area. The highest incidence among PD patients was found for bullous pemphigoid. The incidence of bullous pemphigoid is considerably increased compared to previous reports and reveals regional differences. Further studies are needed in order to clarify these findings.

Bone turnover in hyperthyroidism before and after thyrostatic management.

Hyperthyroidism is associated with enhanced osteoblastic and osteoclastic activity, and patients frequently have low bone mineral density and high bone turnover. The aim of this study was to examine the bone formation and resorption markers trend in 12 female patients, before and after normalization of thyroid activity. The following measurements were made at baseline and 1 and 6 months after hormone normalization induced by methimazole treatment: total alkaline phosphatase (ALP), bone alkaline phosphatase (BALP), collagen type C-terminal propeptide (PICP), osteocalcin (BGP), telopeptide (ICTP), urinary-hydroxyproline/urinary creatinine (uOHP/uCreat), urinary calcium/urinary creatinine (uCa/uCreat) and deoxypyridinoline crosslinks (D-Pyr). Compared with controls, all of these parameters were significantly increased (ALP p = 0.014; BALP p = 0.0001; PICP p = 0.013; BGP p = 0.009; ICTP p = 0.0001; uOHP/uCreat p = 0.002; uCa/uCreat p = 0.044; crosslinks p = 0.0001). After treatment the values of ALP, BALP and PICP in hyperthyroid patients showed an initial slight increase and then a significant downwards trend (ALP p = 0.008, BAP p = 0.001, PICP p = 0.026). Furthermore, resorption markers showed a significant decrease (uOHP/ uCreat p < 0.005 and D-Pyr p < 0.008). As regards lumbar BMD patients, measurements were significantly reduced in comparison with the control group (p = 0.005). Six months after serum thyroid hormones level normalization, we observed a significant increase (p=0.014 vs baseline). Both neoformation and resorption markers are useful to assess pathological bone turnover and bone involvement in hyperthyroidism. They could also be employed to monitor the effect of antithyroid treatment on bone and to indicate if bone antiresorption therapy should be considered.

Hepatitis C infection in an Italian population not selected for risk factors.

AIMS/BACKGROUND: This study estimated the prevalence of HCV infection and relationship with viremia in a general population. The inhabitants of Albavilla town were personally invited to participate. METHODS: Out of 3997 inhabitants falling within the age range 18-85 years, 2403 (participation rate 60.1%) were examined for transaminases, HCVAb, HCVRNA, genotype and immunoblot assay. The following information was collected: sex, age, blood transfusions, surgery, use of glass syringes, drug addiction, alcohol consumption, tattoos and body mass index. RESULTS: 115 (4.8%) were HCVAb+, the prevalence being 1.2% under 40 years. Transfusion in the past was the only risk factor for HCV infection. Among the HCVAb+ subjects, 71 (61.7%) were HCVRNA+. 40.8% of the HCVAb+/HCVRNA+ group had normal ALT, compared with 68% of those with HCVAb+/HCVRNA-. The HCV genotypes in the 71 HCVRNA+ subjects were: 2a/2c in 58 (81.7%), 40% of them with normal ALT;1b in 11 (15.5%), none with normal ALT; genotype 3 in two (2.8%). CONCLUSION: The prevalence of HCVAb in this general population was 4.8%. About 3% were HCVRNA positive and of these genotype 2a/ 2c was present in 81.6%.

Serum cholesterol and chronic hepatitis C.

Total serum cholesterol levels have been studied in 100 patients with histological diagnoses of chronic hepatitis B and 100 wit chronic Hepatitis C, all without cirrhosis, and two age- and sex-matched control groups (B and C). Mean serum cholesterol levels of the groups were compared also in relation to sex, liver function, duration of the disease, alcohol intake, mass index, liver enzymes, presence of liver steatosis and severity of the liver disease on the basis of the histological activity index. The percentages of patients with serum cholesterol level < 150 mg/dl and > 240 mg/dl were also calculated. The mean serum cholesterol level was significantly lower in hepatitis C: 176 md/dl vs 194 mg/dl of hepatitis B (p = 0.004) and 198 of control C (p = 0.000). Twenty eight hepatitis C patients had serum cholesterol < 150 mg/dl vs 10 with hepatitis B (p = 0.001). In multivariate regression analysis, only the type of virus infection was independent related to serum cholesterol level (p = 0.0063).