Case Presentation: A 75-year-old man with a history of glaucoma and diabetes presented to the hospital for evaluation of bilateral painful conjunctivitis, vision loss, abdominal pain and diarrhea with associated 20-pound weight loss over the previous 3-4 months. Testing for Lyme disease, syphilis, Bartonella, Varicella, HIV, and TB were all negative. Rheumatologic workup showed positive HLA-B27 and ANA 1:640, with negative RF, CCP, and ANCA. He was initially diagnosed with atypical HLA-B27 uveitis and saw both ophthalmology and rheumatology within the Veteran Affairs (VA) system. Patient completed a course of steroids and received one dose of Adalimumab without improvement. Due to continued symptoms, he was referred to Ophthalmology at Hershey Medical Center and was directly admitted for expedited inpatient workup. On admission, labs were notable for elevated ESR, CRP, and thrombocytosis. A CT pan-scan revealed a mesenteric mass. Biopsy revealed lymphoplasmacytic infiltrate with storiform fibrosis and IgG4 positive cells – consistent with IgG4-related disease. Rheumatology started patient on treatment with oral prednisone and rituximab infusions. Eyesight returned to 80-90% of baseline functioning at discharge with continued improvement on prednisone eye drops and outpatient rituximab infusions.

Discussion: IgG4-RD is a rarely encountered entity with variable clinical presentation. Disparate symptoms and involvement of any organ system can complicate diagnosis and delay treatment. This patient’s diagnosis was delayed for over 3 months and required inpatient hospitalization when earlier outpatient work-up was possible. Initial treatment for IgG4-RD is high-dose steroids which usually results in rapid improvement1. Other immunosuppressants such as rituximab have been used as well with 95% clinical response rate in one study4. Relapses are common4, however, and further research regarding long-term prognosis is needed.

Conclusions: This case emphasizes the importance of including IgG4-RD in the differential for patients presenting with idiopathic scleritis or in cases of treatment non-responsive scleritis. Furthermore, this case also emphasizes the necessity of obtaining a biopsy, both for pathologic confirmation of IgG4-RD diagnosis as well as to rule out malignancy.