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Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides are infrequent autoimmune diseases characterized by inflammation of the walls of small vessels leading to tissue and endothelial damage. On the other hand, IgG4-related disease is a fibroinflammatory disease characterized histologically by lymphoplasmacytic infiltrates with IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis that may affect nearly every organ of the body. There are similarities in clinical, serological, radiological, and histopathological features between both diseases, and hence, they usually mimic each other complicating the differential diagnosis. Furthermore, reports of patients with the coexistence of both conditions (overlap syndrome) have been reported. We herein report a patient with an unequivocal diagnosis of ANCA-associated vasculitis, specifically granulomatosis with polyangiitis (posterior uveitis, polyneuropathy, pauci-immune glomerulonephritis with crescent formation and granulomas, and MPO-ANCA positivity) and IgG4-related disease (thoracic aortitis, tubulointerstitial nephritis with prominent IgG4+ plasma cell infiltration, fibrosis, and obliterative arteritis, high levels of serum IgG4, and eosinophilia) overlap syndrome., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objectives: To assess the use and causes of withdrawal of glucocorticoids and immunosuppressors among patients with primary Sjögren's syndrome (pSS) in the clinical setting., Methods: We retrospectively reviewed the medical records of 155 pSS patients and registered demographics, glandular/extraglandular features, serological data, cumulative ESSDAI and SSDDI. A single rheumatologist attributed the indication and cause of withdrawal of glucocorticoids and immunosuppressors., Results: 92.2% of the patients were female, mean age 57.4±14.7 years and median follow-up 11 years. One hundred and four (67%) patients received glucocorticoids and/or immunosuppressors: 3.8% only glucocorticoids, 43.9% only immunosuppressors and 56.5% their combination. The most used drugs were antimalarials (46.4%), prednisone (36.7%), azathioprine (AZA) (23.8%) and methotrexate (MTX) (18%). At the multivariate analysis, the presence of non-erosive arthritis OR 5.02 (95% CI 1.74-14.47, p=0.003) and the median cumulative ESSDAI score OR 1.10 (95% CI 1.03-1.17, p=0.002) were associated with the use of these drugs. The causes of withdrawal were: 39% improvement, 35.2% patient's own decision, 18.1% toxicity and 11% lack of efficacy. We found toxicity in 14.2% MTX users, 9.7% for AZA, 9.7% for antimalarials and 7.6% for cyclophosphamide., Conclusions: More than half the patients received glucocorticoids and/or immunosuppressors and a not negligible number decided on their own to suspend them, alerting physicians of secondary adverse events and tolerability.

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.