engleski

Adverse effects of TNF inhibitor in a patient with DiGeorge syndrome and juvenile idiopathic arthritis

Biologics such as tumor necrosis factor alpha (TNF-α) inhibitors are used in the treatment of various inflammatory diseases, principally in patients refractory to standard treatment. Having in mind that introduction of TNF-α inhibitors contributes to increased susceptibility to infections in addition to their effects on the immune system of the patient, with contradictory information about elevated risk of malignancy, their clinical use could be challenging in patients who suffer from immunodeficiency disorders. Case presentation This case describes a medically complex 21-year old fellow with DiGeorge syndrome and IgG and IgA hypogammaglobulinemia underlying juvenile idiopathic arthritis treated with adalimumab and substitution of immunoglobulins, after the failure of nonsteroidal anti-inflammatory drugs and methotrexate. Adalimumab, by blocking TNF-α, moved the cytokine balance toward anti- inflammatory side and decreased inflammatory markers’ plasma levels and clinical signs of synovitis. This condition was favourable for the appearance of herpes zoster as a result of T helper (Th) 1 weakening only two months after the initiation of adalimumab. Prolonged therapeutic Th1 weakening within Th17 orientation caused psoriasis. Thereafter, probably domination of pro-fibrotic factors induced bilateral lung reticulo-nodular infiltrates with the enlargement of mediastinal lymph nodes, which disappeared after empiric antibiotic and immunoglobulin therapy, and discontinuation of adalimumab. TNF-α inhibitor was replaced with methotrexate and soon after with sulfasalazine and low dose of corticosteroids with satisfying effect. Conclusion Frequent regular controls are indispensable for monitoring disease activity, possible side effects of treatment and for adjusting the therapy in patients with JIA and primary immunodeficiency treated with biologic therapy and substitution of immunoglobulins.

TNF inhibitor ; DiGeorge syndrome ; juvenile idiopathic arthritis

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