We compared the presence of diverse cytokines and regulatory T and B cells in skin biopsies of discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). We included 19 patients with DLE, 13 with SCLE, 8 healthy controls, and 5 patients with hypertrophic scars. We assessed the CLASI activity score. To determine IL-22-producing cells and the subpopulation of CD4+/IL-17A+-, CD4+/IL-4+-, and CD4+/IFN-γ +-expressing T cells, CD123+/IDO+ pDCs, CD25+/Foxp3+ Tregs, and CD20+/IL-10+-producing B cells, an immunostaining procedure was performed. Also intracellular IL-22, IL-17, IL-4, IFN-γ, and Foxp3 in CD4 T cells, IL-10 in B cells, and IDO in pDCs were analyzed by flow cytometry in peripheral blood. The main cellular participation in both lupus groups was IL-17- and IL-22-producing cell responses both at skin and at peripheral blood but prevailed in DLE. The CLASI activity scores negatively correlated with Th22 subpopulation and positively correlated with CD25+/Foxp3+ Treg cells. In conclusion a proinflammatory and regulatory imbalance coexists in cutaneous lupus, both responses being more intense in DLE.

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BackgroundPrimary Sjögren's syndrome (pSS) is a multisystem autoimmune disease that encompasses extraglandular features that are traditionally managed with steroids and immunosuppresors.ObjectivesTo assess the use of prednisone (PDN) and immunosuppressors in a retrospective pSS cohort and to evaluate their association with damage accrual.MethodsWe reviewed the medical records of 155 pSS patients who regularly attended our Institution (2010-2014) with at least 6 months of follow-up. We used a standardized form to register demographics, glandular (oral/ocular symptoms, parotid enlargement) and extraglandular features. We specifically reviewed data regarding the use of PDN and immunosuppresors from the diagnosis until the last visit. We also scored the SSDI at the last visit (n=116). We used descriptive statistics, Chi-square test and logistic regression analysis reporting OR and 95%C.I.ResultsPatients were 92.2% females, mean age 57.4±14.7 years and median follow-up 11 years (0.5-47.9). Fifty-one patients (32.9%) were free of treatment and 104 (67%) had ever received PDN and/or immunosuppressors. The latter group presented more parotid enlargement (55.8% vs. 37.3%, p=0.04), lymphadenopathy (34.6% vs. 13.7%, p=0.007), arthritis (45.2% vs. 9.8%, p<0.0001), autoimmune hepatitis (9.6% vs. 0%, p=0.02), pulmonary (16.3% vs. 3.9%, p=0.03) and hematologic involvement (44.2% vs. 25.5%, p=0.03). The percentage of use, median number of use periods, maximum dose, main indication and percentage of toxicity are shown at Table 1. Some patients with isolated parotid enlargement (PDN 3%, AZA 2%, antimalarial 1%, MTX 0.6%) or oral/ocular dryness (PDN 1.9%, antimalarial 7%, AZA and MTX 0.6% for each) also received treatment. The overall median SSDI score was 3 (0-8). Patients with a SSDI>2 (n=60) used more frequently cyclophosphamide (15% vs. 0%, p=0.003) and less often antimalarials (35% vs. 55%, p=0.02). At the regression logistic analysis adjusted by disease duration, a SSDI >2 was associated with the presence of any extraglandular feature (OR 4.4, 95% C.I. 1.39-13.9, p=0.01) whereas the use of antimalarials was protective (OR 0.36, 95% C.I. 0.16-0.82, p=0.01).Table 1DrugFrequency, n (%)Median number of used periodsMaximum doseMain indicationToxicityPDN57 (36%)1 (1–5)60 mg/day (50–100)Combination of diverse extraglandular featuresNot evaluatedMethylprednisolone7 (4)6 (3–11)1 gr/pulseNeurologic involvement0%Methotrexate28 (15)1 (1–2)12.5 mg/week (7.5–20)Arthritis4/28 (14.2%)Azathioprine37 (23.8)1 (1–3)100 m/gday (50–150)Autoimmune hepatitis5/37 (13.5%)Antimalarials72 (48)1 (1–4)Hidroxycloroquine 200mg/day (200–400)Arthritis7/72 (9.7%)Cloroquine 150 mg/day (150–300 mg)Cyclophosphamide13 (8)6 pulses (1–8)1 gr per doseNeurologic involvement1/13 (7.6%)Mycophenolate mofetil2 (1.2)12.5 mg/day (1.5–2.5)Vasculitis0%Rituximab3 (1)2 (1–2)500 mg per doseNeurologic involvement0%ConclusionsMore than 50% of pSS patients received PDN and/or immunosuppresors during their follow-up. This fact confirms the systemic nature of the disease and should al...

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