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

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Abstract

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.

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Authors and Affiliations

  1. 3rd Medical Department (Nephrology and Rheumatology), Sana Klinikum Offenbach, Starkenburgring 66, Offenbach Am Main, 63069, Germany

    Julian Sahm, Judith Schreiber & Kirsten de Groot

  2. Department of Ophthalmology, Goethe-Universität Frankfurt am Main, Frankurt, Germany

    Claudia Grunwald

  3. Institute for pathology, cytology and molecular pathology (ÜGP), Wetzlar, Germany

    Susanne Heib

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  1. Julian Sahm
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Correspondence to Julian Sahm.

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Sahm, J., Schreiber, J., Grunwald, C. et al. Extrapulmonary Sarcoidosis with Enigmatic Manifestations, Delaying Diagnosis and Treatment: Case Report. SN Compr. Clin. Med. 3, 331–339 (2021). https://doi.org/10.1007/s42399-020-00675-5

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