Behçet's Disease: A Rare Cause of Epididymo‐Orchitis

Behçet's disease (BD) is a rare, chronic, remitting and relapsing, inflammatory disorder affecting multiple systems; the skin, joints, eyes, blood vessels, nervous system and the gastrointestinal tract [1]. Epididymis-orchitis (EO) or orchi-epididymitis is a rare but a recognized clinical manifestation of BD [2-4] but has not received attention by researchers and even in the EULAR recommendations for the management of BD and the more recently published British Association of Dermatologists and British Society for Rheumatology living guideline for managing people with Behçet's disease 2024 [5, 6]. EO has been reported in BD with various prevalence; 2% in France, China and USA, around 19% in Turkey, 27% in Jordan and 31% in Iraq [3]. Patients with BD presenting with EO are usually in their second or third decades and have established disease with recurrent painful mouth, genital and scrotal ulcerations, papulopustular rash and large joints arthritis. EO does not seem to be a first or presenting manifestation of BD [3, 4]. The pathergy test and HLA B51 are more likely to be positive. The affected patient presents with acute pain, swelling and tenderness of the testicle and epididymis. Though usually unilateral, both sides can be affected. The patient may be feverish with associated mild constitutional symptoms. Causes of EO vary according to age. In children, mumps is a common cause, while in teenagers and young adults, sexually transmitted diseases are the commonest causative factor. In many patients, the cause is not clear. In older males, bladder outlet obstruction causing urinary stasis and retrograde infection is the commonest cause. Amiodarone has been reported as a cause of EO [7]. Tuberculosis can occur at any age. Inflammation of the blood vessels (vasculitis) of the testes is seen in classic polyarteritis nodosa and rarely in other vasculitic diseases. Neoplastic changes of the testicles if suspected, ultrasound examination, tumour markers and CT chest, abdomen and pelvis are requested. The underlying pathological reason for the development of EO is not clear because of lack of histological studies. One possible explanation is inflammation of the blood vessels. The testicles, epididymis and the spermatic cord are very vascular. Another theory is inflammation of the lining of epididymal tubules. More studies are needed. The diagnosis is confirmed with ultrasound imaging. However, an infective cause needs to be excluded. Examination and culture of urine and urethral discharge are necessary. Blood tests looking for possible tuberculosis or mumps are required if deemed necessary. Patients with EO and BD tend to seek advice from their general practitioner or emergency departments. Most patients may receive antibiotics. The USA Centers for Disease Control and Prevention recommends intramuscular ceftriaxone 500 mg in a single dose plus doxycycline 100 mg orally twice a day for 10 days in sexually active men presenting with EO [8]. Antibiotics can be adjusted to target the causative organism, once microbiological results become available. Doxycycline may help with its additional anti-inflammatory activity. However, colchicine and nonsteroidal anti-inflammatory drugs are usually effective but if the patient has severe symptoms and causes other than BD are excluded, we recommend a short tapering course of oral prednisolone. We have not seen any patient who are on long-term azathioprine or biologic therapy, presenting with EO. The condition settles within a few days to a fortnight but may recur. In resistant and/or recurrent cases, azathioprine needs to be considered. Dapsone has been reported to be an effective treatment [3]. There is no evidence that EO affects fertility in males with BD [9]. The key message is that EO in patients with BD is not rare and needs to be considered in every individual presenting with EO, more so in regions with a high prevalence of the disease. EO is not a first clinical feature of BD but develops after the onset of other features particularly painful mouth and genital ulceration, arthralgia and papulopustular rash. The author has nothing to report. The author declares no conflicts of interest.