FRI0612 IGG4-related disease in eastern mediterranean: clinical features and outcomes of a large cohort

Abstract

BackgroundSince majority of IgG4-related disease (IgG4-RD) patients in the literature are from Far East and United States, there is a lack of large series from other parts of the world.ObjectivesWe aimed to identify the clinical characteristics and outcome of Turkish IgG4-RD patients from a tertiary center.MethodsIn step 1, clinical and histopathological features of patients having any disease under the clinical spectrum of IgG4-RD were retrospectively reviewed. For step 2, patients prospectively diagnosed are enrolled into the study. Patients fulfilling the “definite” diagnosis according to comprehensive diagnostic criteria were recruited (enrolled (n=52), excluded (n=47).ResultsMedian age was 51.1 years and no gender predominance was observed (Male/female: 26/26). Retroperitoneal fibrosis is the most frequent presentation; others were lymphadenopathy, orbital pseudotumor, pancreas and salivary glands in decreasing order (Table). Twenty-four (46.1%) of patients had localized involvement. Corticosteroids were mainstay of treatment in 92.5% of patients, and in 57.5% with any immunosuppressive agents as first line treatment. Rituximab has been used for cases resistant to previous treatment or with relapses in 19 (47.5%) of patients. A complete response was achieved in 52.5% of patients and partial response (<50% of regression) in 40%. Two patients deceased due to IgG4-RD attributed problems and no malignancy was observed (median follow up: 18 months).Table 1.Distribution of clinical findings and organ involvement (n=52)Constitutional symptoms, n (%) Fatigue31 (59.6) Tiredness16 (30.7) Night sweats15 (28.8) Weight loss14 (26.9) Fever13 (25)Retroperitoneal fibrosis, n (%)23 (44.2)Lymphadenopathy, n (%)20 (39.2)Any cardiovascular involvement, n (%)15 (28.8) Periaortitis12 (23.1) Pericardium5 (9.6) Coronary periarteritis4 (7.7) Abdominal aort aneurysm1 (1.9)Orbital pseudotumor, n (%)12 (23.1) Orbitalmass/proptosis6 (11.5) Extraoculary muscles6 (11.5)Pancreas, n (%)12 (23.1)Major salivary glands, n (%)11 (21.2)Lacrimal glands, n (%)9 (17.3)Mediastinal fibrosis, n (%)6 (11.5)Ear, nose, sinuses, n (%)5 (9.6)Lung fibrosis, n (%)5 (9.6)Skin , n (%)4 (7.7)Pleura, n (%)4 (7.7)Gall bladder and Biliary ducts, n (%)4 (7.7)Thyroid, n (%)3 (5.8)Liver, n (%)3 (5.8)Kidney (mass) , n (%)3 (5.8)Pachymeningitis, n (%)2 (3.8)Breast involvement: (n=1), tubulointerstitial nephritis (n=1).ConclusionsWe observed similar features with previous European cohorts however no male predominance was seen. Even though conventional immunosuppressives were used in more than half of patients, treatment had switched to rituximab ∼50% patients owing to resistance or relapses.Disclosure of InterestNone declared