Introduction: Cutaneous vasculitis occurs in a sizable minority of patients with Sjogren syndrome. In addition, their response to different modalities of therapy is variable.
Case Description: We present a case of a 66-year-old female with Sjogren syndrome cutaneous vasculitis in whom combination treatment with Rituximab and Azathioprine failed to show a favorable response. However, methotrexate proved to be an excellent alternative.
Discussion: In cutaneous vasculitis, in addition to the necessary local therapy applied to the affected limbs, methotrexate produced a complete response when other treatment modalities failed. Therefore, it may be advisable to use methotrexate to treat cutaneous vasculitis before trying Rituximab.
Conclusion: In cutaneous vasculitis associated with Sjogren syndrome, methotrexate can be an early effective therapeutic strategy.
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http://dx.doi.org/10.1177/11795476221088489 | DOI Listing |
Background: When Behçet's disease is complicated with gastrointestinal ulcers, it is referred to as intestinal Behçet's disease (BD). Clinically uncommon, this condition can involve the entire gastrointestinal tract, often presenting diagnostic challenges in differentiation from Crohn's disease.
Methods: In this case, atypical BD was diagnosed through endoscopic examination, whereas latent tuberculosis infection (LBTI) was confirmed via T-SPOT and PPD tests.
Turk J Pediatr
December 2024
Department of Pediatric Rheumatology, Umraniye Training and Research Hospital, University of Health Sciences, İstanbul, Türkiye.
Background: Polyarteritis nodosa (PAN) is a rare and serious form of systemic necrotizing vasculitis that predominantly affects medium and small-sized arteries, with central nervous system involvement being particularly uncommon. Treatment strategies are tailored according to the extent and severity of the disease. While conventional therapy includes glucocorticoids and conventional disease-modifying-rheumatic drugs (cDMARDs), biologic agents may be critical for severe and refractory cases.
View Article and Find Full Text PDFBMJ Open
December 2024
INSERM UMR1231 Génétique des Anomalies du Développement (GAD), Université de Bourgogne, Dijon, France.
Introduction: The megalencephaly capillary malformation polymicrogyria (MCAP syndrome) results from mosaic gain-of-function variants. The main clinical features are macrocephaly, somatic overgrowth, neurodevelopmental delay and brain anomalies. Alpelisib (Vijoice) is a recently FDA-approved PI3Kα-specific inhibitor for patients with PIK3CA-related overgrowth spectrum (PROS).
View Article and Find Full Text PDFOrphanet J Rare Dis
January 2025
Department of Rheumatology and Immunology, The First Affiliated Hospital, Fujian Medical University, Fuzhou, 350005, Fujian, China.
Background: Intestinal Behçet's syndrome (IBS) has high morbidity and mortality rates with serious complications. However, there are few specific biomarkers for IBS. The purposes of this study were to investigate the distinctive metabolic changes in plasma samples between IBS patients and healthy people, active IBS and inactive IBS patients, and to identify candidate metabolic biomarkers which would be useful for diagnosing and predicting IBS.
View Article and Find Full Text PDFArch Dermatol Res
January 2025
Department of Internal Medicine, University of Central Florida College of Medicine, Orlando, FL, USA.
Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by pustules that rapidly progress into ulcers that commonly affect the lower limbs. Recently, successful treatment of PG has been reported with anti-IL 17 treatments. However, there have also been several reports of "paradoxical" induction of new PG lesions after use of IL-17 inhibitors.
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