engleski

Simultaneous treatment with TNF inhibitor and IVIG in a patient with primary immunodeficiency and autoimmunity: a treatment conundrum

Background and aims: DiGeorge syndrome (DGS) is a primary immunodeficiency disease which can generate underdeveloped B lymphocytes, causing hypogammaglobulinemia in 6% of patients. Autoimmune diseases are recurrent among these patients, with juvenile idiopathic arthritis (JIA) being 50 times more common. Our aim is to present a patient suffering from DGS and JIA treated simultaneously with TNF inhibitor and IVIG, which is a rare occurrence in clinical practice. Methods: All medical information was obtained from patient’s medical records from 2009 to 2017 at the University Hospital Centre Zagreb, Croatia. Results: Male patient was diagnosed with rheumatoid factor negative polyarticular JIA at the age of 3. The following years he was continuously treated with NSAIDs, methotrexate and corticosteroids. Due to frequent infections, dysmorphic facial structures, agenesis of left kidney and ventricular septal defect, DGS was diagnosed at the age of 15. On the account of underdeveloped B lymphocytes, the patient received IVIG every 4-6 weeks. A year later, due to remitting relapses of polyarthritis, adalimumab was introduced. He was successfully treated for a two-year period after which he developed psoriasis, mediastinal lymphadenopathy and interstitial lung infiltrates, which subsided when adalimumab was revoked. Conclusion: The introduction of anti-TNF therapy was debatable due to the added risk of infections and malignancies, to which the patient was already susceptible on the account of the DGS. However, concurrent therapy with IVIG and TNF inhibitors can be beneficial but it is of great importance to carefully monitor the patient for possible side effects and interactions between drugs.

primary immunodeficiency ; DiGeorge syndrome ; autoimmunity ; TNF inhibitor ; IVIG

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