Male hypogonadism in a patient with Cogan syndrome

To the Editor: Cogan syndrome is a rare, rheumatic disease characterized by inflammation of the ears and eyes that may also be associated with vasculitis in other areas of the body.¹ Lunardi and colleagues advanced identification of the eye, inner ear, nervous system and endothelium autoantigens of Cogan syndrome with their finding that the isolated 12-residue Cogan peptide is homologous to four autoantigens: laminin, connexin 26, the collagen disease-associated Ssa/Ro, and the receptor-like phosphatase DEP-1/CD148, and one viral protein.² The condition can lead to visual difficulty, hearing loss and dizziness. It is fatal in less than 10% of patients. Mild disease may be treated with anti-inflammatory medications, including steroids and nonsteroidal anti-inflammatory drugs.¹

The age-related decrease in testosterone and potential hypogonadism may result in decreased libido and erectile dysfunction, loss of muscle mass and strength, weight gain, and declining cognitive function.^3–5 Men on long-term corticosteroid therapy often have low testosterone levels.⁶ Androgen receptor levels are regulated by androgens, other steroids and non-steroidal hormones.⁷ Therefore, we thought it would be interesting to highlight a case of male hypogonadism in a patient with Cogan syndrome.

A 47-year-old male with increasing loss of sexual drive, general lack of enthusiasm, and frequent exhaustion presented to our department because of loss of libido and erectile dysfunction for one year. He was divorced without children. Four years previously, a diagnosis of Cogan syndrome was made based on a bilateral progressive hearing loss, tinnitus and ataxia, as well as a bilateral interstitial keratitis following a viral infection of the upper airways. He had previously been treated with methylprednisolone for approximately 4 years with a maintenance dose of 4 mg daily. Physical examination revealed sparse secondary hair, muscle atrophy, a normal prostate and testis with a volume of 8 mL on both sides. All baseline hematological, biochemical and additional hormone parameters where within normal values except for total cholesterol 9.3 mmol/L (normal range, 0.0.–5.0 mmol/L), HDL cholesterol 1.2 mmol/L (normal range, 1.0.–5.0 mmol/L), LDL cholesterol 6.7 mmol/L (normal range, 0.0.–3.0 mmol/L) and triglyceride 3.1 mmol/L (normal range, 0.0.–1.7 mmol/L). His total testosterone levels were 8.4 and 13.5 nmol/L (normal range, 10.5–49 nmol/L), free testosterone 170 pmol/L (normal range, 174–900 pmol/L), luteinizing hormone 15.7 U/L (normal range, 1.5–5.0 U/L), follicle-stimulating hormone 38.9 U/L (normal range, 1–10.5 U/L). A semen analysis showed azoospermia. Chromosome analysis excluded abnormalities, so the karyotype was 46,XY. Testicular biopsy confirmed marked hyalinization and thickening of the basement membrane of the spermatic tubules. Color duplex sonography of the aorta and intraabdominal vessels excluded severe vascular manifestations of Cogan syndrome. Osteodensitometry revealed a normal bone density for age. Therapy with intramuscular injections of testosterone enanthate 250 mg was started every 3 weeks and atorvastatin 20 mg daily was added. At last review, lipids were well controlled. Corticosteroids have a catabolic effect, often intensified by the androgen deficiency. Contrary to the fact that long-term therapy with corticosteroids can induce secondary hypogonadism our patient developed primary hypogonadism. Testicular biopsy excluded vasculitis as an underlying pathological mechanism. Our case is a clear example of a rare systemic autoimmune disease that requires a broad multidisciplinary approach to optimize its treatment and improve the patient’s well-being.