Hypogonadotropic Hypogonadism and Juvenile Idiopathic Arthritis in an African Boy: What is the Pathophysiological Link?

The association between hypogonadotropic hypogonadism and juvenile chronic arthritis has rarely been reported in the literature. We report an untreated case of systemic juvenile idiopathic arthritis in a young African male with co-presentation of hypogonadotropic hypogonadism. Possible pathophysiological and etiological links are discussed. A 16-year-old boy was received in our outpatient department for chronic arthritis with temporomandibular involvement and fever. There was no family history of rheumatic diseases or psoriasis. Body temperature was 39.5°C at admission. The clinical examination found synovitis of wrists and knees and inflammatory lymphadenopathy. This polyarthritis occurred in a context of hypogonadism marked by impuberism of Tanner classification stage P2G2. Laboratory tests showed biological inflammatory syndrome and hyperferritinemia with collapsed glycosylated ferritin at 11%. Hormonal testing found low blood testosterone (0.08 mg/L) and pituitary hormone levels attesting to hypogonadotropic hypogonadism. Screening for infections was negative. The immunological assessment for antinuclear antibodies, rheumatoid factor, and anti-cyclic citrullinated peptide antibodies were negative. Standard radiography showed bilateral wrist carpitis. The olfactory bulb was present and normal by cerebral magnetic resonance imaging. The diagnosis of systemic juvenile idiopathic arthritis associated with hypogonadotropic hypogonadism, probably related to delayed puberty, was retained. A therapy combining corticosteroid, methotrexate for arthritis, and hormone replacement with testosterone led to regression of arthritis, biological inflammatory syndrome, and hypogonadism. The presence of rheumatic disease in this context of hypogonadism, regardless of its cause, is mainly associated with very low testosterone levels and the presentation of arthritis in these patients tends to be more severe.