Eyes are a window to the body: A journey from subconjunctival hemorrhage to SLE and inferior vena cava stenting
Year: 2024
Type: article
Study design: Case Report
Abstract: A 45-year-old female presented to eye emergency with a complaint of sudden onset redness in the left eye (OS) for 2 days. Her visual acuity was 6/6 in both eyes (OU). Anterior segment examination revealed subconjunctival hemorrhage (black arrow) in Fig. 1a in OS, and the rest of the anterior segment examination was normal in OU. Dilated fundus examination revealed white linear deposits (black arrow) in the lower temporal third order arterioles with no clinical sign of occlusion in the right eye (OD) [Fig. 1b] and similar white linear deposit in the third order arterioles (black arrow) with an area of retinal whitening (red arrow) with superficial hemorrhages signifying vascular occlusion temporally in OS [Fig. 1c].Figure 1: (a) Black arrow depicts subconjunctival hemorrhage. Blue arrow depicts malar rash. (b) Black arrow depicts arteriolar occlusion. (c) Black arrow shows arteriolar occlusion and red arrow area of whitening or retinal ischemia. (d) Red arrow shows caput medusae. (e) and (f) Yellow arrow shows areas of capillary non-perfusion and green arrow shows areas of collateral formationWhat is Your Next Step? Blood pressure Ask h/o trauma or other systemic illness Fundus fluorescein angiography systemic examination/investigations All Findings The blood pressure was 140/110 mm hg. History of fever and pain abdomen was there, but there was no history of ocular trauma. Caput medusae or dilated abdominal veins [Fig. 1d], ascites, and hepato-spleenomegaly were present. Malar rash was present [Fig. 1a, blue arrow]. Fundus fluorescein angiography revealed widespread areas of peripheral non-perfusion (yellow arrows) corresponding to areas supplied by the arterioles with white linear deposits suggesting sites of arteriolar occlusion and collateral formation (green arrows) compensatory mechanism of the body to ischemic insult in OU [Fig. 1e and f]. On computed tomography (CT) angiography of the abdomen, she had esophageal varices and inferior vena cava thrombosis. On systemic work-up, her antiphospholipid antibody (APLA), anti-nuclear antibodies (ANA), and IgG B2microglobulin came positive. She underwent inferior vena cava stenting. Diagnosis Secondary APLA syndrome due to systemic lupus erythematosus (SLE) with Budd Chiari syndrome. Correct answer: E Discussion Anti-phospholipid syndrome (APLS) is an auto-immune multi-system disorder characterized by auto-antibodies directed against phospholipid-binding proteins. The syndrome can be primary without any other systemic disease or secondary; that is, part of SLE. APLS can lead to pulmonary embolism, acute coronary syndrome, mesenteric thrombosis, or hepatic venous-occlusive disease. To prevent any thrombotic or embolic phenomenon in patients of APLS, the anti-coagulation regimen should be very clearly defined.[1] Budd-Chiari syndrome is characterized by ascites, hepatomegaly, abdominal pain, varicose veins, and collaterals over the anterior abdominal wall because of obstruction of the hepatic veins or the inferior vena cava; its association with APLS secondary to SLE is rare.[2] Anti-phospholipid antibody (APLA)-positive patients are predisposed to the formation of antigen antibody complexes that lead to clot formation in arteries and veins. These might have embolized and led to BRAO in these patients.[3] Conclusion Complete ocular examination is must. This case also highlights the importance of full ocular and systemic examination that led to life-saving treatment. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.
Source: Indian Journal of Ophthalmology
Authors: Manu Sharma, Shilpa Viswanath, Ramandeep Singh
Language: English
Subfield: Rheumatology
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4402902882