Churg-Strauss syndrome (CSS) is a rare, systemic, antineutrophil cytoplasmic antibody–associated vasculitis involving small and medium-sized vessels that affects multiple tissues, particularly the lungs. Ophthalmologic involvement includes keratitis, conjunctival nodules, dacryoadenitis, myositis, uveitis, retinal vascular occlusion, and cranial neuropathies such as optic neuropathy.1 Transient monocular vision loss (TMVL) in CSS and the diagnosis of CSS by temporal artery biopsy (TAB) are both rare. We report a case of TMVL due to CSS vasculitis diagnosed by TAB. Report of a Case A 56-year-old man with a history of asthma had shortness of breath and 3 episodes of TMVL in the right eye, which he described as a “web that gradually coalesced into a white veil.” Each episode lasted approximately 2 minutes before spontaneously resolving. Other symptoms included cough, headache, and mild fever for 2 weeks. Examination revealed hypoxia, wheezing, bibasilar lung rales, atrial fibrillation, and scattered erythematous skin plaques and macules. His blood leukocyte count was 15 200/μL (to convert to ×109 per liter, multiply by 0.001), his erythrocyte sedimentation rate was 38 mm/h, and his C-reactive protein level was 68 mg/L (to convert to nanomoles per liter, multiply by 9.524). Although prior chest radiographs and computed tomography had shown patchy, nonfixed pulmonary infiltrates, his radiograph on admission showed only left lower-lobe pulmonary atelectasis (Figure 1A). Head computed tomography revealed extensive ethmoid sinus opacification and sphenoid sinus mucosal thickening, similar to a previous scan (Figure 1B). Head and neck magnetic resonance angiography and brain magnetic resonance imaging findings were unremarkable. View LargeDownload Figure 1. Radiographic findings of a 56-year-old man with Churg-Strauss syndrome diagnosed by temporal artery biopsy. A, Radiograph showing left lower-lobe atelectasis. B, Noncontrast computed tomography showing respiratory sinus opacification. Respiratory decompensation prompted treatment with broad-spectrum intravenous antibiotics. During his admission, heparin was administered because he had 6 more episodes of TMVL, which were thought to be transient ischemic attacks associated with atrial fibrillation. Although his ophthalmic examination findings were normal, his elevated C-reactive protein level raised concern for possible giant cell arteritis, for which oral prednisone treatment was started and a TAB was performed. Histopathologic analysis revealed active, circumferential arteritis with numerous eosinophils, mononuclear leukocytes, and karyorrhectic debris concentrated in the intima and adventitia but also involving the media (Figure 2A). Intimal damage was marked with loss of elastica in areas of intense intimal inflammation where mural thrombi containing numerous eosinophils deposited along the lumen of the vessel were present (Figure 2B). Focal fibrinoid necrosis was seen in a branch artery (Figure 2C). These findings, together with the patient's history of asthma and eosinophilia, suggested the diagnosis of CSS vasculitis. Histopathologic review of a prior skin punch biopsy revealed perivascular dermatitis with numerous eosinophils (Figure 2D), consistent with this diagnosis. His clinical, radiographic, and pathologic findings confirmed the diagnosis.2 View LargeDownload Figure 2. Churg-Strauss syndrome histopathologic findings (original magnification ×200). A, Temporal arterial transmural leukocytic infiltrate with chiefly intimal damage (hematoxylin-eosin). B, Elastica damage beneath eosinophilic mural thrombosis (Verhoeff–Van Gieson stain). C, Fibrinoid necrosis of the temporal arterial branch (hematoxylin-eosin). D, Perivascular dermal leukocytic infiltrate containing eosinophils near a sweat gland (hematoxylin-eosin). The TMVL episodes ceased, pulmonary function improved, and eosinophilia resolved after the patient began prednisone therapy. He was transitioned to treatment with methotrexate, and anticoagulation was discontinued. Antineutrophil cytoplasmic antibody testing results were negative. Comment A TAB is almost always performed when giant cell arteritis is suspected, but it sometimes results in a diagnosis of a systemic necrotizing vasculitis. In a prospective study of elderly patients with presumed giant cell arteritis, 4.5% of all positive biopsies demonstrated a non–giant cell vasculitis.3 Cases with TMVL in which TAB leads directly to the diagnosis of CSS are exceedingly rare.4 The American College of Rheumatology diagnostic criteria for CSS include asthma, blood eosinophilia greater than 10%, paranasal sinusitis, pulmonary infiltrates, histologic proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. These criteria yield a sensitivity of 85% and a specificity of 99.7% when 4 are present, and our patient met 5 of the 6 criteria. We suspect that the mechanism of TMVL in our patient was secondary to reversible thromboses developing along the surface of the intima, which showed intense inflammation and fibrin deposition. The histopathologic findings we show, without vessel occlusion from intimal hyperplasia, appear to be at the earliest stage reported for a TAB with CSS vasculitis. The TAB may be diagnostic for several types of systemic vasculitis. This case demonstrates that vasculitis other than giant cell arteritis should be considered in patients with TMVL and elevated acute-phase reactants. Back to top Article Information Correspondence: Dr Elner, Kellogg Eye Center, University of Michigan, 1000 Wall St, Ann Arbor, MI 48105 (email@example.com). Conflict of Interest Disclosures: None reported. References 1. Kubal AA, Perez VL. Ocular manifestations of ANCA-associated vasculitis. Rheum Dis Clin North Am. 2010;36(3):573-58620688251PubMedGoogle ScholarCrossref 2. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33(8):1094-11002202307PubMedGoogle ScholarCrossref 3. Généreau T, Lortholary O, Pottier MA, et al; French Vasculitis Study Group. Temporal artery biopsy: a diagnostic tool for systemic necrotizing vasculitis. Arthritis Rheum. 1999;42(12):2674-268110616017PubMedGoogle ScholarCrossref 4. Buhaescu I, Williams A, Yood R. Rare manifestations of Churg-Strauss syndrome: coronary artery vasospasm, temporal artery vasculitis, and reversible monocular blindness: a case report. Clin Rheumatol. 2009;28(2):231-23319034601PubMedGoogle ScholarCrossref
JAMA Ophthalmology – American Medical Association
Published: Jan 1, 2013
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