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Adrenal and Gonadal Hormone Variations during a Febrile Attack in a Woman with Tumor Necrosis Factor Receptor-Associated Periodic Syndrome

Laboratory of Neuroendocrinoimmunology (R.H.S., P.H., J.S.), Department of Internal Medicine I, Division of Rheumatology, University Hospital, 93042 Regensburg, Germany; Department of Medicine V (H.-M.L.), Division of Rheumatology, University of Heidelberg, 69115 Heidelberg, Germany; and Section of Immunobiology (M.K.), Yale University School of Medicine, New Haven, Connecticut 06520

Address all correspondence and requests for reprints to: Rainer H. Straub, M.D., Professor of Experimental Medicine, Laboratory of Neuroendocrinoimmunology, Department of Internal Medicine I, University Hospital, 93042 Regensburg, Germany. E-mail: rainer.straub{at}klinik.uni-regensburg.de.

Context: TNF-receptor-associated periodic syndrome (TRAPS) is a hereditary fever syndrome that results from mutations in the TNF-receptor superfamily 1A gene (TNFRSF1A). It is characterized by periodic fever, arthralgia, abdominal pain, myalgia, headache, and skin lesions.

Objective: Because adrenal and gonadal hormone cascades are modulated by TNF, this study aimed to investigate specific hormones and enzyme steps during an attack phase in a woman with TRAPS.

Design: Morning blood samples were taken from a 38-yr-old woman before, during, and after the febrile episode in the late luteal, menstrual, and early follicular phase of the menstrual cycle, respectively.

Results: Serum cortisol levels were markedly increased throughout the entire observation period and demonstrated a dip during the attack phase. In contrast, serum levels of dehydroepiandrosterone and 17-hydroxyprogesterone demonstrated a sharp rise during the febrile episode. Dehydroepiandrosterone in relation to androstenedione or cortisol was increased. Indicative of aromatase activation, estrone and 17ß-estradiol demonstrated a marked increase during the attack phase.

Conclusion: This study suggests that some important steroid hormone-conversion steps are activated (aromatase) and inhibited (second step of the P450c17 and the 3ß-hydroxysteroid dehydrogenase) during the inflammatory attack phase in a TRAPS patient. These changes of enzyme pathways are typical on the basis of increased TNF signaling.