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FRI0612 IGG4-related disease in eastern mediterranean: clinical features and outcomes of a large cohort
  1. O Karadag1,
  2. A Erden1,
  3. AS Ayhan2,
  4. EC Bolek3,
  5. U Kalyoncu1,
  6. B Armagan1,
  7. A Sari1,
  8. L Kilic1,
  9. A Akdogan1,
  10. T Hazirolan4,
  11. B Akdogan5,
  12. SA Bilgen1,
  13. DE Baydar2,
  14. S Kiraz1,
  15. I Ertenli1
  1. 1Department of Rheumatology
  2. 2Department of Pathology
  3. 3Department of Internal Medicine
  4. 4Department of Radiology
  5. 5Department of Urology, Hacettepe University Faculty of Medicine, Ankara, Turkey

Abstract

Background Since 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.

Objectives We aimed to identify the clinical characteristics and outcome of Turkish IgG4-RD patients from a tertiary center.

Methods In 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).

Results Median 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)

Conclusions We 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 Interest None declared

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