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Arch Ophthalmol. 2004;122:393-396.

A case of orbital myositis in a patient with Churg-Strauss syndrome (CSS) is reported herein. To our knowledge, this association has been reported only once previously.¹ We also describe the previously reported ophthalmic manifestations of CSS.

Report of a Case
A 55-year-old woman was referred to our unit with a 1-week history of redness and pain behind the right eye, which worsened on eye movement.

Her medical history included adult-onset, steroid-dependent asthma, diagnosed at 30 years of age and currently stabilized with inhaled corticosteroid use. She also had, from allergic rhinitis, nasal polyps and severe sinus disease requiring repeated surgery. She had been noted to have peripheral blood eosinophilia on several occasions for the last 7 years and had a recurrent rash for the last 4 years involving her trunk and back, responsive to corticosteroid treatment and found to be interstitial granuloma annulare on tissue biopsy results obtained 4 years earlier. A repeated biopsy 1 year later revealed an intense urticarial inflammatory reaction with perivascular eosinophilic and lymphocytic inflammatory infiltrates. Peripheral blood eosinophil levels, 4 and 3 years previously, were raised at 2300/µL and 2060/µL, respectively. A bone marrow biopsy obtained 3 years earlier revealed a marrow eosinophilia and an elevation of eosinophil precursor populations (eosinophil and eosinophil precursors, 10%) with normal erythropoiesis, myelopoiesis, marrow cells, and marrow architecture. Findings from bone marrow lymphocyte surface marker analysis and gene rearrangement studies were normal. Allergies included sulfur, aspirin, and certain foods, for which she had undergone desensitization therapy.

On examination, she had swelling and tenderness of the right upper lid and bony tenderness over the right cheek. Her right conjunctiva was inflamed and edematous with a raised lesion on the inferior aspect (Figure 1A). She had 2 mm of right axial proptosis (Hertel exophthalmometer readings of 16 mm OS and 14 mm OD, respectively) and a red, swollen, tender, right superior rectus muscle insertion (Figure 1B). The right medial and lateral recti insertions also appeared prominent. Visual acuity (using meters) was 6/6 OD and 6/5 OS. Findings from a full, dilated ocular examination of both eyes was otherwise unremarkable.

View larger version (160K): [in this window] [in a new window] Figure 1. A, Clinical photograph showing an inflamed and edematous right conjunctiva, with a raised nodule on the inferior aspect. B, Clinical photograph showing a red, swollen right superior rectus insertion.

A computed tomography scan of the orbits showed significant enlargement of the right superior rectus muscle along with some enlargement of the other recti. There was also opacification of all paranasal sinuses bilaterally (Figure 2A). Biopsy findings of the right conjunctival lump were confirmed histologically to be a focal eosinophilic inflammatory cell infiltrate with no granulomata or evidence of vasculitis (Figure 2B). Complete blood cell count findings were normal, with no evidence of eosinophilia. Rheumatoid factor was borderline at 20 kIU/L (reference, <20 kIU/L). Other unremarkable results included serum electrolyte levels; liver function tests; thyroid function tests; serum angiotensin-converting enzyme, antinuclear antibody, and antineutrophil cytoplasmic antibody levels; chest x-ray; echocardiography; barium swallow; and stool examination.

View larger version (226K): [in this window] [in a new window] Figure 2. A, Computed tomography coronal view of the orbits, revealing enlargement of the right-sided recti musculature, especially superior and lateral recti. Paranasal sinuses are opacified bilaterally. L indicates left. B, Histologic specimen. Conjunctival nodule biopsy revealed a marked eosinophilic infiltrate (hematoxylin-eosin, original magnification x100).

She began a reducing course of oral prednisolone (initial dose, 60 mg), 1 day prior to undergoing conjunctival biopsy, and her myositis resolved during the following 2 weeks.

Five months after her episode of myositis, she underwent a biopsy of nasal tissue during functional endoscopic sinus surgery for ongoing sinusitis. The biopsy findings revealed nonspecific inflammatory nasal polyps with prominent eosinophilia. Again, no granulomatous inflammation or vasculitis was identified. These results were considered consistent with CSS, however nondiagnostic in a pathological sense.

Comment
Churg-Strauss syndrome is an uncommon, systemic, vasculitic disorder and is largely typified by a history of asthma, allergic disease not including drug allergy, and eosinophilia (10%) on differential white blood cell count.² Churg-Strauss syndrome classically affects the lung and paranasal sinuses, but extrapulmonary manifestations, namely skin, cardiac, and gastrointestinal, are also commonly described. Ocular features are unusual.¹ Early recognition of the ocular features of this syndrome, and early institution of appropriate treatment, may minimize complications and potentially completely reverse the disease process.

We report a case of orbital myositis in a patient with CSS. Our patient meets the American College of Rheumatology 1990 criteria for CSS, with a documented history of asthma, eosinophilia (25% differentiated white blood cells), and allergic disease, namely, allergic rhinitis, pansinusitis, and food allergy.² Conjunctival biopsy revealed florid extravascular infiltration of eosinophils; however, there were no granulomas or vasculitis. Myositis was diagnosed on clinical and radiological grounds. The conjunctival lump and the superior rectus myositis arose simultaneously and, therefore, can be considered the same disease process, particularly so because both the conjunctival lesion and myositis resolved concurrently with the administration of oral steroids.

The eosinophilic tissue infiltrative phase of CSS is well described, and the lack of histological evidence of granulomas or vasculitis may occur with biopsy techniques such as fine-needle aspiration or bronchoalveolar lavage. However, other explanations include disease suppression by corticosteroid use, or a prevasculitic phase of the illness. A forme fruste type of CSS is described, in which the disease has been partially or completely suppressed by systemic or inhaled corticosteroid therapy for asthma and only appears clinically when changes in steroid therapy are made.³ This is illustrated by our case, in which the biopsy did not reveal any evidence of granulomata or vasculitis in a patient taking inhaled corticosteroids, with a history of oral steroid use, and 1-day use of oral prednisolone. The rapid response to corticosteroids, as seen in this case, is typical of CSS.^3-4

Ocular features of CSS in the literature are relatively sparse but varied. These include conjunctival nodules,^{1, 5-6} corneal ulcer,⁷ episcleritis,⁵ uveoscleritis,^{5, 7} ischemic optic neuropathy, amaurosis fugax,^{1, 6-7} central retinal vein occlusion, central and branch retinal artery occlusion, retinal vasculitis,¹ retinal hemorrhage,⁷ cranial nerve palsies,^{1, 7} and orbital inflammatory syndrome.¹

A review by Takanashi et al¹ describes 15 cases in the literature that meet the American College of Rheumatology 1990 criteria for the classification of CSS. This includes a case of dacryoadenitis and myositis. On initial examination, this patient had proptosis and radiologically confirmed symmetrical lacrimal gland and superior and lateral rectus muscle swelling. Biopsy findings of the lacrimal gland and lateral rectus muscle revealed extravascular infiltration of eosinophils.

Churg-Strauss syndrome may be difficult to diagnose.^{2, 4} The combination of asthma, eosinophilia, and a history of allergies must raise the suspicion of CSS and the possibility of vasculitis. However, biopsy of ocular tissue may give somewhat indeterminate results.^{1, 6}

To our knowledge, orbital myositis has only once been previously reported.¹ In conclusion, we present a case of superior rectus muscle myositis due to CSS. The concurrent association with an eosinophilic conjunctival nodule further illustrates the heterogeneity of the ocular manifestations of CSS.

AUTHOR INFORMATION

Kathryn Billing, MBBS; Raman Malhotra, FRCOphth; Dinesh Selva, FRACS, FRANZCO; Thomas Dodd, FRCPA
Adelaide, Australia

Corresponding author: Dinesh Selva, FRACS, FRANZCO, Oculoplastic and Orbital Clinic, Department of Ophthalmology, Royal Adelaide Hospital, North Terrace, Adelaide, Australia 5000 (e-mail: Awestwoo{at}mail.rah.sa.gov.au).

REFERENCES
1. Takanashi T, Uchida S, Arita M, Okada M, Kashii S. Orbital inflammatory pseudotumor and ischaemic vasculitis in Churg-Strauss syndrome: report of two cases and review of the literature. Ophthalmology. 2001;108:1129-1133. FULL TEXT | ISI | PUBMED 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;33:1094-1100. ISI | PUBMED 3. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). 1984;63:65-81. PUBMED 4. Churg A. Recent advance in the diagnosis of Churg-Strauss syndrome. Mod Pathol. 2001;14:1284-1293. FULL TEXT | ISI | PUBMED 5. Robin JB, Schanzalin DJ, Meisler DM, et al. Ocular involvement in the respiratory vasculitides. Surv Ophthalmol. 1985;30:127-140. PUBMED 6. Nissim F, Von der Valde J, Czernobilsky B. A limited form of Churg-Strauss syndrome. Arch Pathol Lab Med. 1982;106:305-307. PUBMED 7. Weinstein JM, Chui H, Lane S, et al. Churg-Strauss syndrome (allergic granulomatous angiitis): neuroophthalmologic manifestations. Arch Ophthalmol. 1983;101:1217-1220. ABSTRACT

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