Pulmonary Mycobacterium avium complex infection with vascular Ehlers–Danlos syndrome: A case report

Abstract

A female patient developed multiple intestinal perforations at 31 and 43 years of age. Because of her family history of pneumothorax and intestinal perforation, Ehlers–Danlos syndrome (EDS) was suspected when she visited our hospital at 52 years. She was diagnosed with vascular Ehlers–Danlos syndrome (vEDS) and developed bilateral external iliac artery dissection. A CT scan at the time of admission revealed granular and infiltrative shadows in both lungs with bronchiectasis. The patient was also diagnosed with Mycobacterium avium complex (MAC) pulmonary disease, and drug susceptibility to clarithromycin was confirmed. After treatments with rifampicin, ethambutol, and clarithromycin were started, the acid-fast bacilli cultures taken from sputum were negative, and respiratory symptoms partially improved after about 1 month. vEDS is reportedly associated with lung diseases, such as pneumothorax and cystic lung lesions, but there are few reports of respiratory infections with vEDS. Moreover, there are no reports of complications associated with MAC disease. We report a case of vEDS with rare complications and suggest the possible mechanism of infection.

Keywords

• Vascular Ehlers–Danlos syndrome
• COL3A1
• Mycobacterium avium complex
• Pneumothorax