Multiple autoimmune syndrome complicating the management of diabetic retinopathy

PURPOSE: To describe a case of multiple autoimmune syndrome presenting with type I diabetes, choroidal vitiligo, coeliac disease, pseudohypoparathyroidism, and immune thrombocytopenia purpura (ITP), the latter diagnosed seven years after the initial presentation. OBSERVATIONS: A 26-year-old female presented with bilateral severe diabetic retinopathy. Panretinal photocoagulation (PRP) was initially declined due to poor adherence to treatment. Thirty-three months after the initial presentation, a progression of the retinal disease to bilateral proliferative retinopathy, macular edema, and epiretinal membranes was noted. Additionally, an ischemic branch retinal vein occlusion was diagnosed in the inferior nasal quadrant of the left eye. Over this period visual acuity declined from 6/9 bilaterally to 6/24 and 6/30 in the right and left eyes, respectively. PRP was then performed under subtenons anesthesia. Excessive hemorrhage was noted from the site of the conjunctival wound, and Tranexamic acid was prescribed postoperatively. Investigations did not reveal a primary coagulopathy. Seven years after the initial presentation, the patient was admitted to hospital with a spontaneous right frontal lobe intracerebral hemorrhage, from which a recovery occurred without neurologic deficit. Hematological parameters remained normal for this admission and the cause of the spontaneous hemorrhage remained undiagnosed. Seven months after this episode, the patient was admitted to the Hematology ward after a five-week history of gingival hemorrhage subsequent to a dental procedure. As the platelet count was 16 × 10(9)/L, a diagnosis of ITP was confirmed. However, the platelet count failed to respond to treatment with Prednisone, intravenous Immunoglobulin, Tranexamic acid, Eltrombopag, and Rituximab. A second fatal intracranial hemorrhage occurred two months later. CONCLUSION AND IMPORTANCE: Multiple autoimmune syndrome may complicate the presentation and management of diabetic retinopathy. In some cases, the manifestations of systemic autoimmune disease may dominate the clinical picture. Management of the more complex disease burden, in this case, became an increasingly perplexing multidisciplinary predicament with each additional autoimmune disorder diagnosed over the treatment course.