Orbital Manifestation of Immune Reconstitution Inflammatory Syndrome in Whipple’s Disease

Figure

Magnetic resonance imaging skull and orbita.

Following 16 years of treatment with steroids, azathioprine, methotrexate, and infliximab for “seronegative polyarthritis, malabsorption and lymphadenopathy”, a 51-year-old male patient was diagnosed with Whipple’s disease and treated with ceftriaxone and co-trimoxazole in accordance with the guidelines. Rheumatic and intestinal symptoms subsequently receded, and the PCR for Tropheryma whipplei in the small bowel turned negative. Right-sided exophthalmos developed 7 months later in the setting of severe pain and a rectal temperature of 39.5 C ˚. Laboratory tests yielded a leukocyte count of 30 000/mL and a C-reactive protein (CRP) value of 90 mg/dL. Magnetic resonance tomography (Figure) showed protrusio bulbi and scleritis posterior including the retrobulbar space and the optic nerve sheath as well as thickened ocular muscles. The risk for the development of immune reconstruction inflammatory syndrome (IRIS), which accompanies antibiotic-only treatment in patients with Whipple’s disease following discontinuation of pretreatment with immunosuppressants, was not known at the time. Today, steroids would be given prophylactically alongside antibiotics in an attempt to prevent IRIS and, in this case, blindness.

Prof. Dr. med. Gerhard E. Feurle, DRK Krankenhaus Neuwied, [e-mail]

Dr. rer. nat. Verena Moos, Medizinische Klinik I CBF, Charité, Berlin

Conflict of interest statement: The authors declare that no conflict of interests exists.

Translated from the original German by Christine Rye.

Cite this as: Feurle GE, Moos V: Orbital manifestation of immune reconstitution inflammatory syndrome in Whipple’s Disease.
Dtsch Arztebl Int 2022; 119: 734. DOI: 10.3238/arztebl.m2022.0205