Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome: Report of two cases and review of the literature

doi:10.1016/S0161-6420(01)00557-7

Ophthalmology

Volume 108, Issue 6, June 2001, Pages 1129-1133

https://doi.org/10.1016/S0161-6420(01)00557-7 Get rights and content

Abstract

Objective

To clarify the characteristics of ocular manifestations in Churg-Strauss syndrome (allergic granulomatosis and angiitis).

Design

Two interventional case reports and literature review.

Participants

Two patients with Churg-Strauss syndrome with ocular manifestations are described; 15 previously reported cases and the present 2 cases of Churg-Strauss syndrome with ocular manifestations are reviewed.

Intervention

Ocular manifestations were divided into two groups: orbital inflammatory pseudotumor and ischemic vasculitis.

Main outcome measures

The onset, conjunctival involvement, orbital imaging, antineutrophil cytoplasmic antibodies (ANCA), and visual prognosis were evaluated.

Results

The characteristics of the orbital inflammatory pseudotumor type (eight cases) are chronic onset, positive conjunctival involvement, abnormalities in orbital imaging studies, negative ANCA, and good visual prognosis. The ischemic type (nine cases) is characterized by sudden onset, no conjunctival involvement or abnormalities in imaging studies, positive ANCA, and occasional poor visual prognosis.

Conclusions

Orbital inflammatory pseudotumor and ischemic vasculitis may represent two essential characteristics of Churg-Strauss syndrome, granulomatosis and angiitis, respectively. The clinical features of the two types are so distinct that differentiation may be meaningful for diagnosis and treatment of Churg-Strauss syndrome with ocular manifestations.

Section snippets

Case 1

A 43-year-old Japanese man with a 20-year history of exophthalmos and paranasal sinusitis first experienced a mild fever, cough, and pharyngeal pain on July 3, 1997. He was seen at the Department of Internal Medicine of Kurashiki Central Hospital on July 6. Moist rales were heard on auscultation. Chest roentgenogram showed miliary infiltrates in the left lower lung. Decreased arterial O₂ and increased arterial CO₂ were measured (PaO₂, 49.2 mmHg; PaCO₂, 37.3 mmHg; normal values, 69 to 116 and 32

Discussion

The first case in this study presented the chronic dacryoadenitic form of orbital inflammatory pseudotumor that was demonstrated by MRI. Orbital inflammatory pseudotumor is reportedly associated with tissue eosinophilia and eosinophil degranulation that induce inflammation of the orbital tissues and lead to fibrotic process.22 Extravascular eosinophil infiltrates in the lacrimal grand and the lateral rectus muscle in this case are compatible with the diagnosis of orbital pseudotumor. The second

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¹: The authors have no proprietary interest in any aspect of this study.

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Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome: Report of two cases and review of the literature1

Abstract

Objective

Design

Participants

Intervention

Main outcome measures

Results

Conclusions

Section snippets

Case 1

Discussion

Rev Med Interne

Am J Ophthalmol

Am J Ophthalmol

Clin Neurol Neurosurg

Ophthalmology

Rheum Dis Clin North Am

Nomenclature of systemic vasculitides. Proposal of an international consensus conference

Arthritis Rheum

Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases

Mayo Clin Proc

Churg-Strauss vasculitis presenting with severe visual loss due to bilateral sequential optic neuropathy

Br J Ophthalmol