Overlapping Case of Advanced Systemic Sclerosis and IgG4-Related Disease after Autologous Hematopoietic Stem Cell Transplantation

We report the case of a 44-year-old female patient with overlapping progressive diffuse SSc and sinonasal IgG4-RD. After 11 years of ineffective SSc treatment, AHSCT was performed. The 63-month follow-up showed a regression of SSc symptoms. AHSCT was not intended as treatment in the case of IgG4RD, although the first symptoms of the disease developed before transplantation. The sinus lesions progressed after AHSCT and remained indolent only after surgical treatment (bilateral ethmoidectomy, sphenoidotomy, intranasal buccal antrostomy), which allowed histopathological confirmation of IgG4-RD.PMID:38541223 | PMC:PMC10972323 | DOI:10.3390/medicina60030496

Source: Medicina (Kaunas) - March 28, 2024 Category: Universities & Medical Training Authors: Alisa Julija Dulko Irena Butrimiene Alma Cypiene Valdas Peceliunas Donatas Petroska Ernesta Stankeviciene Rita Rugiene Source Type: research