Ashley McCain, od hughes Syndrome: Diagnosing the Darkness



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Ashley McCain, OD

Hughes Syndrome: Diagnosing the Darkness



  1. Case History

  • 25 year old Navajo female referred from ER

  • Complains of flashing lights and darkening of vision for one week in the right eye

  • ER doctor is concerned vision changes are secondary to hydroxychloroquine use

  • Medical history of Lupus, Hughes syndrome, severe Raynauds , Rheumatoid arthritis

  • Medications: Amlodipine 2.5 mg tablet daily, APAP/Oxycodone 325/5mg prn for pain, Multivitamin daily, Celecoxib 200mg daily, Cyclobenzaprine 10 mg at bedtime, Ferrous Sulfate 325 mg daily, Hydroxychloroquine 200 mg daily, Fluoxetine 20 mg twice a day, MS Contin 30mg daily, Prednisone 20mg daily, Promethazine 25 mg daily, Sildenafil 20 mg three times a day, Trazodone 25 mg at bedtime, Omeprazole 20 mg daily.

  • Patient is a smoker

  • Denies oral contraceptive use



  1. Pertinent Findings

  • Entering visual acuities without correction 20/40 OD, 20/50 OS. Pinhole 20/25 OD, 20/20 OS

  • SLEX: unremarkable

  • Nerve C/D ratio of 0.35 round OU, pink and distinct with no evidence of edema

  • No embolus noted in vessels OU

  • Macula is flat and distinct OU

  • Retinal periphery is flat and intact 360 degrees OU

  • No evidences of holes/breaks/tears OU

  • Due to age and symptoms of amaurosis fugax labs are ordered same day

  • Lab results: ESR>140, Low HGB , Low HCT, Elevated WBC with left shift

  • MRI and CTA ordered the next day following lab results

  • MRI reveals 8.7mm mass adjacent to right optic nerve



  1. Differential Diagnosis

  • Cerebral vasculitis – leading

  • Thrombosis secondary to Hughes syndrome

  • Vasospasm secondary to Raynauds

  • Induced amaurosis fugax secondary to mass on right optic nerve



  1. Diagnosis and Discussion

  • Patient was diagnosed with inadequate coagulation secondary to Antiphospholipid Antibody syndrome (Hughes syndrome), an orbital apex lesion of the right optic nerve, microcystic anemia , thrombocytosis, and lupus nephritis

  • This case is very unique. The patient has several autoimmune disorders that correlate to one another. Initial referral from ER appeared to be for a retinal detachment or tear. Further lab work revealed underlying systemic inflammation due to autoimmune disease. The patient was non-compliant with prednisone dosage in the past and stated she had been tapering off the medication. She was no longer taking her daily aspirin for blood thinning. On top of these conditions the MRI also revealed a small mass that appeared to be impinging on the right optic nerve. Patient was referred to St. Joseph hospital in Phoenix where further imaging and testing was conducted. The small mass was determined to be a mucous retention cyst medial to the right orbital apex. The cyst was not compressing the optic nerve and was determined not to be the etiology of the amaurosis fugax.



  1. Treatment, management

  • Prednisone 60 mg daily (patient previously uncompliant)

  • Aspirin 325 mg daily

  • Re-initiated Mycophenalate 250 mg, 6 capsules two times a day

  • Referred patient to Rheumatologist to co-manage

  • Referred patient to neuro-ophthalmology for further evaluation of mass



  1. Conclusion

  • This case was very complex and illustrates the correlation between systemic autoimmune diseases and the eye. Amaurosis fugax is one of the most common ocular symptoms associated with Hughes syndrome. Eye care clinicians should be aware when to refer for further testing. Life-long oral anticoagulation is the treatment of choice to prevent future reoccurrences and thrombosis.



  1. Bibliography

  • Lally L, Sammaritano L. Vasculitis in Antiphospholipid syndrome. Rheum Dis Clin N AM. 2015(41):109-123

  • Petri M. Review of Classification Criteria for Systemic Lupus Erythematosus. Rheum Dis Clin N AM. 2005(31):245-254

  • Boey M, Colaco CB, Gharavi AE, Elkon KB,Loizou, GR V Hughes. Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating lupus anti-coagulant. British Medical Journal. 1983(287): 1021-1023

  • Atanasovva P. Antiphospholipid syndrome and Vascular Ischemic (Occlusive) Diseases: An Overview. Yonsei Med J. Dec 31, 2007; 48(6):901-926

  • Saxena R, Mahajan T, Mohan C. Lupus nephritis: current update. Arthritis Res Ther.2011; 13(5):240.



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