No abstract

Changes in nailfold capillaroscopy in systemic sclerosis patients could be related to the disease severity. The aim of this study was to investigate whether patients with "late" scleroderma (SD) pattern have more organ involvement than patients with "early/active" SD pattern. Forty-six Argentinian patients (44 women and 2 men), with a diagnosis of systemic sclerosis, were distributed in two groups based on the presence of late and early/active patterns. Organ involvement was assessed as follows: pulmonary function by chest radiography, high-resolution chest tomography (HRCT), lung volume tests, and diffusing capacity for carbon monoxide (DLCO); esophageal involvement by manometry; and pulmonary arterial hypertension (PAH) by Doppler echocardiography and six-minute walk test. Honeycombing of the lungs evaluated by HRCT was more frequently present in patients with late pattern compared with early/active patients (p = 0.01). We also found statistically significant differences in lung volume tests (p = 0.03) and DLCO (p = 0.02) between the two SD pattern groups. Esophageal manometry showed a significantly higher frequency of motility disorders in the group with late pattern (p = 0.0024). In this study, patients with late pattern had higher frequency of pulmonary and esophageal involvement compared with patients with early/active pattern.

Background Sjögren’s Syndrome is the most common connective tissue disease that appears in association with other autoimmune diseases. Objectives To determine if significant differences exist in the clinical, immunological and histological profile between patients with pSS with an associated organ-specific autoimmune disease (AAD) and patients without it. Methods It was analyzed the data included in the GESSAR’s database. Eight organ-specific autoimmune diseases were selected: Autoimmune Thyroiditis (AT), Primary Biliary Cirrhosis (PBC), Autoimmune Hepatitis (AH), Celiac Disease (CD), Primary Sclerosing Cholangitis (PSC), Ulcerative Colitis (UC), Crohn’s Disease (CR) and Vitiligo (VT). Two groups of patients with pSS were obtained: one with organ-specific AAD and another one without it. The following variables were analyzed: 1-Associated organ-specific autoimmune disease, 2-clinical profile (gland and extra-gland manifestations), 3-immunological profile (ANA, Rheumatoid Factor, anti Ro, anti La, cryoglobulinemia, hypergammaglobulinemia, hypocomplementemia) and 4-histological profile (minor salivary gland (MSG) biopsy 3-4 degree according to Chishom-Mason classification). It was obtained the prevalence of each variable for each group and the differences between the groups were analyzed. Results 285 patients were included, 95.9% were female, with a median age of 57.5 (RIC 47-66) and a median age at the time of the pSS diagnosis of 52 (RIC 39-62). Ninety patients (31.57%) suffered from at least one associated organ-specific autoimmune disease. The most frequent disease was AT, followed by PBC, AH, CD, UC and VT in frequency order. Neither cases of CR nor PSC were found. Regarding the clinical profile, the articular involvement was the most frequent, with no differences between both groups. The respiratory involvement was significantly more frequent in the group with AAD (34.44% vs 19.48%; p 0.0096). There were found no differences in the rest of the clinical manifestations as well as in the immunological and histological profile. Conclusions In this study we observed that the patients with pSS and organ-specific AAD presented more frequency of respiratory involvement, which could be due to the inclusion of xerotrachea among the respiratory manifestations. On the other hand, there was no evidence of more prevalence of sicca symptoms, alterations in the immunological profile or in the biopsy of MSG in these patients, therefore the associated autoimmune disease would not have influence over these variables. Despite patients with pSS should be monitored for organ-specific AAD due to their high frequency of association. Disclosure of Interest: None Declared