IgG4-Related disease (IgG4-RD) is a rare systemic autoimmune_x000D_ fibroinflammatory condition, characterized by the development of fibrotic masses_x000D_ and organ dysfunction. In this doctoral thesis, IgG4-RD was studied in Spanish_x000D_ patients for the first time creating a nationwide registry, based on the international_x000D_ consensus criteria on IgG4-RD pathology. The first study showed that patients_x000D_ with IgG4-RD were predominantly middle-aged men. The disease involved_x000D_ multiple organs in 47% of the cases. The most frequently involved zones were_x000D_ retroperitoneum, orbit, salivary glands and pancreas. After completing the first_x000D_ treatment, 39% of the patients had flares. The second study found that only 55%_x000D_ of the participants had serum IgG4 levels over 135 mg/dL. The most commonly_x000D_ used treatments (73% of all) were glucocorticoids (GC), GC with surgery, GC with_x000D_ azathioprine, and surgery alone. They all had a high response rates, but relapses_x000D_ were frequent. The modified IgG4-responder index (IgG4-RI) corresponded to the_x000D_ disease’s activity. No patients developed cancer in the first year after the_x000D_ disease’s diagnosis. In conclusion, IgG4-RD characteristics in Spain were similar_x000D_ to the ones described in the literature. Serum IgG4 elevation was lower than in_x000D_ Asian populations. Treatment with GC was the most frequently used, but disease_x000D_ modifying antirheumatic drugs could be useful. Modified IgG4-RI was useful to_x000D_ monitor the outcomes. The incidence of malignancy was low.
MALALTIA RELACIONADA AMB LA IgG4
Fernández Codina, A. (Author). 12 Mar 2019
Student thesis: Doctoral thesis
Student thesis: Doctoral thesis