Extrapulmonary Sarcoidosis with Enigmatic Manifestations, Delaying Diagnosis and Treatment: Case Report

The following report presents a 30-year-old male with an exceptional course of systemic sarcoidosis. He showed multiple organ threatening manifestations such as aortitis, which led to an aortic replacement-procedure twice, as well as asymptomatic neurosarcoidosis, severe chorioretinitis and vertebral granuloma. Complete diagnostic evaluation has been failed initially. After 4 years of exclusive prednisolone therapy, we reassessed the patient and initiated additional azathioprine-therapy. Chronic sarcoidosis reappeared with severe ophthalmic progression which required TNF-α blockade. Infliximab followed by adalimumab administration led to decline of manifestations of sarcoidosis as seen in follow-up cranial MRI-studies, by regressive chorioretinitis in both eyes and permanent low levels of serological markers of inflammation. Chronic sarcoidosis can be a severe systemic inflammatory disease that can involve any organ. Complete diagnostic evaluation including biopsy is necessary for a suitable treatment. Although there are no randomized controlled trials, we can confirm that TNF-α-agents can be a suitable treatment for severe chronic sarcoidosis.