CASE REPORT: IGG4-RELATED DISEASE IN A PATIENT WITH SJÖGREN SYNDROME, PSORIATIC ARTHRITIS AND AUTOIMMUNE HEPATITIS

• DOI: 10.47660/cbr.2023.2151
• Publication Date: 2024-01-04T07:21:05Z
• Authors: Laura Marcon Bisc...; Luciana Machado K...; Leonardo André Denti; Mateus Camozzato ...; Letícia Fioravant...; Maria Lucia Lemos...; Tatiana Freitas T...; Rafael Coradin; Lilian Scussel Lo...; Thiago Willers
• Keywords: Rheumatoid factor; Autoimmune Hepatitis

Immunoglobulin G4 Related Disease (IgG4RD) is a fibroinflammatory immune-mediated condition that affects multiple organs.Typical presentations of this disease include type 1 autoimmune pancreatitis, sclerosing cholangitis, involvement the major salivary glands or sialadenitis, periorbital disease, and retroperitoneal fibrosis, commonly involving periaortitis.In study, we describe an atypical case IgG4RD. CASE REPORTMale patient, 65 years old, farmer, with previous diagnosis Psoriatic Arthritis (history sacroiliitis, peripheral arthritis, enthesopathy dactylitis), prior use Methotrexate posterior treatment abandonment; Sjögren's Syndrome xerostomia xerophthalmia, exams ANA >1:1280 nuclear fine speckled, Rheumatoid Factor, positive anti-Ro anti-La) for about 10 years.The patient was hospitalized due to insidious onset constitutional symptoms (recurrent fever, night sweats, nausea, lack appetite, fatigue) imaging tests abdominal thoracic lymph node enlargement.Admission showed normocytic normochromic anemia, leukocytosis without left shift, C-reactive protein > 100, decrease in C3 complement -70 (VR 90-180), proteinogram hypergammaglobulinemia -4.18 (51%) high IgG values -4.744 (RV 650-1600) IgE -340 (RV< 158).CT scans noticed enlargement, diffuse peritoneal thickening micronodular infiltration omental adipose planes.A biopsy periportal nodes performed, demonstrating exuberant sinus plasma cell histiocytosis, cells IgG4.Corticosteroid therapy prednisone 40mg/day initiated, significant improvement reduction enlargement new CT scans, discharged outpatient follow-up.