Severe Vaso-occlusive Retinopathy as the Primary Manifestation in a Patient with Systemic Lupus Erythematosus

Abstract

Severe vaso-occlusive retinopathy as the initial manifestation of systemic lupus erythematosus (SLE) is rare. We report a 16-year-old female who developed bilateral visual impairment. Fundus examinations showed bilateral "cherry-red spot" appearance, multiple confluent cotton wool spots, and widespread arterial occlusion. Laboratory studies showed leukopenia, antinuclear antibody (+), and anti-double-stranded DNA antibody (+). Malar rashes, oral ulcers, and bilateral knee joint tenderness were noted during physical examination. SLE was diagnosed and pulse therapy started immediately. Best corrected visual acuity of the left eye improved to 6/10 after treatment. However, there was no visual improvement in the right eye. Four months later, bilateral panretinal laser photocoagulation was performed due to retinal neovascularization. However, tractional retinal detachment of the right eye and vitreous hemorrhage of the left eye still occurred. After undergoing cryoretinopexy of the right eye and intravitreous tissue plasminogen activator injection of the left eye, the visual acuity of the patient's right eye remained hand movement only at 10 cm, but that of the left eye returned to 6/10. The ocular and systemic conditions were stable in the follow-up period of more than 2 years. This case demonstrates that in patients with severe vaso-occlusive retinopathy, a generalized immunological disorder, like SLE, should be suspected.

Vomiting and diarrhea in a woman with systemic lupus erythematosus

“…Lupus mesenteric vasculitis (LMV) results from immune complex deposition and thrombosis of the intestinal vessels in SLE. 1,2 LMV is an uncommon condition in SLE patients with acute abdominal pain. 3 Symptoms of LMV are usually nonspecific and abdomen ultrasonography is very useful for early and correct diagnosis of this uncommon cause of abdominal pain in patients with SLE.…”

Bilateral Central Retinal Artery Occlusion in a Patient with Systemic Lupus Erythematosus

“…1 Ocular involvement is present in about 15% of patients with SLE and may be a presenting feature of SLE. [2][3][4][5][6] Ocular involvement in SLE include keratoconjunctivits sicca, scleritis, uveitis, ischemic optic neuropathy, and occlusion of retinal vessels potentially resulting in blindness. 5 Lupus-associated retinopathy often parallels the severity of systemic inflammation indicating poor disease control.…”

Catastrophic Antiphospholipid Antibody Syndrome and Cocaine Abuse Associated With Bilateral Retinal Vascular Occlusions

“…Previous reports [6][7][8][9] have associated bilateral CRVO with Waldenstrom's macroglobulinemia, hyperhomocysteinemia, and other hypercoagulable states. Additionally, simultaneous CRVO and central retinal artery occlusion have been associated with systemic lupus erythematosus, [10][11][12][13][14] syphilis, 15 Behcet disease, 16 cat scratch disease, 17 septic cavernous sinus thrombosis, 16 infective endocarditis, 16 temporal arteritis, 18 Churg-Strauss syndrome, 19 thrombotic thrombocytopenic purpura, 20,21 leukemia and lymphoma, 16,22 orbital inflammatory pseudotumor, 23 posterior scleritis, 21 AIDS, 24 and APAS. [12][13][14][25][26][27][28][29][30] To our knowledge, there are no other cases of bilateral central retinal artery and vein occlusion reported with APAS or catastrophic APAS.…”