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Year : 2013  |  Volume : 61  |  Issue : 7  |  Page : 365-366

Ocular manifestations of the antineutrophil cytoplasmic antibody and antiphospholipid syndromes

Department of Allergy and Immunology, Apollo Gleneagles Hospital, Kolkata, India

Date of Web Publication30-Jul-2013

Correspondence Address:
Sujoy Khan
Department of Allergy and Immunology, Apollo Gleneagles Hospital, 58 Canal Circular Road, Kolkata - 54
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.111127

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How to cite this article:
Khan S. Ocular manifestations of the antineutrophil cytoplasmic antibody and antiphospholipid syndromes. Indian J Ophthalmol 2013;61:365-6

How to cite this URL:
Khan S. Ocular manifestations of the antineutrophil cytoplasmic antibody and antiphospholipid syndromes. Indian J Ophthalmol [serial online] 2013 [cited 2023 Dec 8];61:365-6. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?2013/61/7/365/111127


I read with interest the article by Kumar Saurabh and colleagues on the patient profile of retinal vasculitis from Eastern India, [1] and would like to comment on the importance of a more comprehensive laboratory work-up to ensure that the vasculitides syndromes associated with significant morbidity and mortality are not missed. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) and antiphospholipid antibody syndrome (APS) both can initially present with only ocular manifestations such as uveitis and work-up to exclude these vasculitides is more important in younger patients without known systemic risk factors. [2],[3]

The AAVs include Wegener's granulomatosis (cytoplasmic staining pattern seen on immunofluorescence of ethanol-fixed human neutrophils, cANCA, with specificity for proteinase 3 [PR3]) while microscopic polyangiitis and Churg-Strauss syndrome (usually have a perinuclear pattern on immunofluorescence, pANCA, with specificity for myeloperoxidase [MPO]) can present with various ophthalmic manifestations. But in a subset of patients, these findings may be the earliest indicators of systemic disease (up to 50% in children). [2],[4] Orbital and anterior segment findings are most common, whereas posterior segment complications such as retinal vasculitis and optic neuropathy occur much less frequently. However, a study on patients with pANCA-associated vasculitis reported that ocular surface (scleritis and peripheral keratitis) and posterior segment manifestations (central or branch retinal vein occlusion, optic neuropathy, acute posterior multifocal placoid pigment epitheliopathy) were the important eye presentations. [5] However, a significant proportion of patients will have systemic symptoms, but may not initially present, and the finding of abnormal chest X ray or serum creatinine or glomerulonephritis should warrant an urgent laboratory request to exclude an ANCA-associated vasculitis.

The autoimmune disease APS is characterized by the consistent presence of antiphospholipid antibodies (or anticardiolipin or anti-β2GPI antibodies) at 12 weeks, arterial or venous thromboses and repetitive foetal loss. Diluted Russel viper venom time (dRVTT) is often used as one of the phospholipid-dependent tests that shows a prolonged APTT which gets corrected only with increased phospholipid concentrations (suggesting the presence of lupus anticoagulant). This can be present without ds-DNA antibodies with a nonspecific positive antinuclear antibody (ANA) only, and hence the addition of lupus anticoagulant in the diagnostic criteria of systemic lupus erythematosus. Ocular involvement in APS occurs in 8-88% of patients, of which 75% with ocular presentation were women with mean age at 40 years. [3] Ocular findings in APS include anterior uveitis, unilateral or bilateral blurring of vision, transient scotoma, uniteral amaurosis fugax (bilateral suggests CNS ischemia), visual field defects and various retinal pathologies (hemorrhages, vasculitis, retinopathy) discussed by Utz VM and Tang J in their review. [3]

Clinicians should therefore have a high index of suspicion of AAV and APS, in especially younger patients, which would be the first step in avoiding the inevitable complications of arterial inflammation and visual loss. Emphasis to lower additional risks of thrombosis such as treating cardiovascular risk factors (hypertension, diabetes, hyperlipidemia, tobacco use or obesity), during immobilization or malignancy, treating microalbuminuria, screening for inherited thrombophilias, and counseling regarding oral contraceptive use remain equally important.

  References Top

Saurabh K, Das RR, Biswas J, Kumar A. Profile of retinal vasculitis in a tertiary eye care center in Eastern India. Indian J Ophthalmol 2011;59:297-301.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
Kubal AA, Perez VL. Ocular manifestations of ANCA-associated vasculitis. Rheum Dis Clin North Am 2010;36:573-86.  Back to cited text no. 2
Utz VM, Tang J. Ocular manifestations of the antiphospholipid syndrome. Br J Ophthalmol 2011;95:454-9.  Back to cited text no. 3
Levi M, Kodsi SR, Rubin SE, Lyons C, Golden R, Olitsky SE, et al. Ocular involvement as the initial manifestation of Wegener's granulomatosis in children. J AAPOS 2008;12:94-6.  Back to cited text no. 4
Matsuo T. Eye manifestations in patients with perinuclear antineutrophil cytoplasmic antibody-associated vasculitis: Case series and literature review. Jpn J Ophthalmol 2007;51:131-8.  Back to cited text no. 5


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