Central Retinal Arterial and Venous Occlusion in a Patient with Systemic Sclerosis and a Review of the Related Literature

Autor: Hiroko Konishi, Kimimasa Nakabayashi, Rie Tagaya, Ippei Doi, Aya Nakase, Aya Mori, Masayoshi Iwakami
Rok vydání: 2021
Předmět:
Zdroj: American Journal of Internal Medicine. 9:214
ISSN: 2330-4316
DOI: 10.11648/j.ajim.20210904.19
Popis: Systemic sclerosis (SSc) is known to cause serious complications at an advanced stage. However, serious ocular complications such as central retinal artery occlusion (CRAO) and/or central retinal vein occlusion (CRVO) have only been reported in a few cases to date. A 51-year-old man with severe Raynaud’s phenomenon, scleroderma up to the forearm and a negative anti-nuclear antibody (ANA) finding developed pulmonary fibrosis (PF) and was admitted to the hospital for the treatment of dyspnea under the diagnosis of SSc. A transbronchial lung biopsy performed 10 days after admission demonstrated mucoid intimal thickening (MIT) of the small arteries in the peripheral lung tissue and the bronchial wall. Prednisolone (PSL), ciclosporin and nintedanib were prescribed for PF treatment but failed to induce any apparent improvement in dyspnea. Finally, methylprednisolone pulse therapy (1.0 g, 3 times) and intravenous cyclophosphamide (IVCY, 500 mg, 6 times) therapy were instituted with a subsequent decrease in the patient’s Krebs von den Lungen 6 (KL-6) and surfactant protein D (SP-D) levels. During IVCY therapy, the patient experienced blurred vision in the right eye, and this was diagnosed as being due to CRAO and CRVO. However, the patient did not manifest any embolic, coagulopathic, or vasculitic symptoms, and there were no abnormal laboratory data suggesting these diseases in relation to this symptom. Therefore, the ocular occlusion was presumed to be due to MIT of small arteries, in addition to severe Raynaud’s phenomenon. Thus, we report the findings of this important case, despite the fact that it lacks a retinal pathology.
Databáze: OpenAIRE