Hepatitis C Virus Induced Sjogren Syndrome - Clinical and Imaging Features.

Currently incurable, Charcot-Marie-Tooth (CMT) disease is the most commonly inherited neurological disorder, which affects a small percentage of the population. The most common cause of CMT is the duplication of a region on the short arm of chromosome 17, which includes the gene PMP22. We report a thirty-seven-year-old man with CMT disease having sleep, memory and attention disorders characterized by brief retrograde amnesia at early age. The patient has no genetic disease in the family, but was diagnosed with diabetes mellitus, which emphasizes the sensory loss and prolonged infections. Diabetes mellitus emphasizes the sensory symptomatology and predisposes to the development of infections with delayed healing.

Contribution of Nail Fold Videocapillaroscopy in Patients with Early Inflammatory Arthritis.

BACKGROUND: Early inflammatory arthritis (EIA) a condition defined by joint swelling, with or without morning stiffness, and/or swelling of metacarpophalangeal and/or metatarsophalangeal joints. Nail fold video-capillaroscopy (NVC) is important for the evaluation of microcirculation in vivo. There are limited data about the role of NVC in EIA. OBJECTIVES: To study the capillaroscopic pattern in patients with EIA. PATIENTS AND METHODS: 27 patients with EIA - 21 women, 6 men, mean age of 41.6±4.2 yrs, mean disease duration of 6.9±3.1 months. Anamnesis and clinical examination were perform to identify clinical signs associated with connective tissue diseases. All the patients were nonsmokers and had no personal medical history of diabetes or non-immune mediated occlusive vascular disease. Blood and urine samples were collected for bio-chemical and immunological evaluation. All the patients were interrogated by NVC. RESULTS: Raynaud's phenomenon was found in 9 patients, puffy fingers in 2 pts, telangiectasia in 2 pts, Sicca symptoms in 6 pts, malar rash in 2 pts, photosensitivity and psoriasis plaque in one patient. Combined information from clinical exam, NVC and immunological assessment allowed a specific diagnosis in 5 patients. CONCLUSIONS: Nail fold capillaroscopy assessment can provide further information and has diagnostic value in some cases of early arthritis. Nail fold capillaroscopy assessment contribution to the differential diagnosis in patients with early arthritis is sometimes significant, especially in poorly clinical and immunological defined cases.

Impaired arterial stiffness in systemic lupus ertythematosus - correlations with inflammation markers.

BACKGROUND: Systemic lupus erythematosus (SLE) is an inflammatory disease caused by autoimmune dysregulation, which mainly affects young women, usually free from atherosclerosis. Accelerated atherosclerosis is a well established complication of SLE and it cannot be explained by Framingham risk factors alone, and has been attributed to complex interactions between traditional risk factors and factors associated with the disease per se, or its treatment. Arterial stiffness and endothelium function may serve as a valuable measure to be counted in the follow-up of these patients prior to a potential cardiovascular event. The aim of the study was to evaluate atherosclerosis, inflammatory process, immune mediated, using imaging techniques and to identify the role of molecules known to be involved in inflammation, hsCRP, homocysteine, IL-6, ESR and fibrinogen, in the development and perpetuation of atherosclerosis in patients with systemic lupus erythematosus. Methods Our prospective study included 53 patients diagnosed with systemic lupus erythematosus and fulfilled the revised ACR (American College of Rheumatology) criteria for the classification of SLE. Exclusion criteria were <18 years of age, history of CVD, infections, diabetes mellitus, dyslipidemia. RESULTS: We enrolled 53 patients with SLE, 50 (94%) women and 3 (6%) men, with a mean age of 31,92 years (SD 5,55; limits 22-44) with no significant difference between sex (31,65±3,4 years in women and 37,33±4,05 years in men). The measurement of inflammation markers revealed increased values for all the variables: ESR had a mean value of 69,19± 14,18mm, fibrinogen 445,66 ±4,56mg%; IL-6 had a mean value of 11,209 ±1,56pg/ml; homocysteine 17,721±2,5374 µmol/l and for hs CRP the mean value was 3,493±1,12 mg/l. The assesement of arterial stiffness showed a mean value of 23,32% (SD 5,82; 95%CI 21,716 - 24,925) for AIx and 9,1m/s (SD 0,49; 95%CI 8,971 - 9,244) for cfPWV. There was a positive, significant correlation between AIx and hsCRP (r=0,612; 95%CI 0,4104 - 0,7576; p<0,001), (r=0,526; 95% CI 0,2979 to 0,6971; p=0,0001), for AIx and homocysteine (r=0,526; 95%CI 0,2979 to 0,6971; p=0,0001). The correlation coefficient with AIx was similar for ESR and fibrinogen (r=0,63 and 0,60). IL-6 and AIx correlated correlated positively, (r=0,369; 95%CI 0,1097 - 0,5813), statistically significant (p=0,006), but the correlation was not powerful. hsCRP and cfPWV were related (r=0,652; 95%CI 0,4677-0,7862; p<0,001); cfPWV also correlated with IL-6 (r=0,6552; 95%CI 0,4677- 0,7862; p<0,0001), homocysteine (r=0,9174; 95%CI 0,8606- 0,9517; p<0,0001), ESR (r=0,74) and fibrinogen (r=0,64). CONCLUSIONS: In summary, our data suggest that arterial stiffness is related to the level of systemic inflammation, and that inflammation is involved in the early alteration of arterial wall. Increase in arterial stiffness can be detected by applanation tonometry, and may serve as an important predictor of future cardiovascular events, since an early diagnosis may have a significant value in preventing the development of major vascular disease.

The outcome of patients with lupus nephritis and the impact of cardiovascular risk factors.

BACKGROUND: Systemic lupus erythematosus (SLE) is the prototype of autoimmune connective tissue diseases. Renal disease is a frequent manifestation of SLE that influences the outcome of the patients. The aim of the current study was to determine and analyze the clinical features and subsequent outcome of 70 patients with LN, followed in our department over the past 5 years, focusing on the impact of cardiovascular risk factors in the renal outcome and mortality. PATIENTS AND METHODS: Our prospective study included 70 patients with SLE and LN and 70 patients with SLE without signs of renal involvement, all patients fulfilled the revised ACR (American College of Rheumatology) criteria for the classification of SLE. Demographical data, risk factors and comorbidities were recorded. RESULTS: Patients with lupus nephritis had a mean age of 37 years (range 15-65, SD 1.8). During the study, we had a rate of drop off of 15 patients with lupus nephritis (21%) and 19 patients without nephritis (26%). Patients with LN had a higher prevalence of positive anti-dsDNA antibodies (85.4% vs 49%, p<0.001, RR=2.2) and a lower percent of rheumatoid factor (FR) positive (5.45% vs 15.68%, p=0.03, RR=0.34) compared with the controls, a higher prevalence of corticosteroid treatment (65.45% vs 7.83%, p<0.001, RR=2.1) and immunosuppressive treatment (AZA 27.27% vs 3.92%, p=0.01, RR=1.71, CFM 34.54% vs 0%, p<0.001, RR=2.16), a higher frequency of hypertension (47.27% vs 9.8%, p<0.001, RR=2.4), hyperlipidaemia (49.09% vs 1.96%, p<0.001, RR=1.81) and anti-PL antibodies (49.09% vs 20%, p=0.001, RR=2.70),and a higher mortality (16% vs 2%, p=0.02, RR=1.76). 20 patients (36.36%) from the survival group (55 patients), evoluated to renal failure, 9.09% of these with end -stage renal failure, results that are similar with the ones in other studies. CONCLUSIONS: The study reveals the fact that cardiovascular risk factors such as hypertension, hyperlipidaemia and antiphospholipid syndrome are associated with a higer rate of mortality and an evolution to end-stage renal disease.

The study of angiogenesis in early rheumatoid arthritis--clinical, immunohistochemical and immunological correlations.

OBJECTIVE: The objective of the study was to analyze several immunohistochemical, histological, and morphometrical aspects of angiogenesis in early rheumatoid arthritis synovium. We aimed to identify possible correlations between the histological and immunohistochemical patterns and the serum levels of VEGF, as well as with clinical and biological markers of disease activity. METHODS: 35 patients with early rheumatoid arthritis below 12 months from the onset, naive for DMARDs, underwent clinical standard examination as well as serum determinations for CRP, RF. anti-CCP2 antibodies and VEGF. DAS28 value has been determined for each patient in order to assess the disease activity. We performed biopsy sampling through arthroscopy, the synovium fragments beeing histopathologically processed, in order to elaborate a total histological score. Immunohistochemical analysis has been performed with quantification of synovial VEGF, VEGF-R1 and CD34 expression. Standard and activated microvascular density (sMVD and aMVD) have been evaluated through double immunostaining (CD34/ VEGF-R1). RESULTS: VEGF and VEGF-R1 have been identified with high prevalence in endothelial cells, in lining and sublining synovial cells, as well as in inflammatory cells. The study focuses on the analysis of aMVD, a valuable parameter, representative for active angiogenesis, which proved to correlate significantly with the serum levels of VEGF, the composite histological score as well as with VEGF-R1 and DAS28. CONCLUSION: The statistic analysis of the data support VEGF-R1 and aMVD as markers with predictive value regarding activity and progression in early stages of rheumatoid arthritis. The validation of preliminary conclusions oblige to continuous research through extending the study group and inclusion of several others biomarkers involved in synovial angiogenesis.