ChurgStrauss syndrome presenting with visual loss
J. Carmichael,
M. Conron1,
H. Beynon1,
J. Crow2 and
T. T. Warner
University Department of Clinical Neurosciences,
1 Department of Rheumatology and
2 Department of Pathology, Royal Free Hospital and University College Medical School, Pond Street, London NW3 2QG, UK
SIR, ChurgStrauss syndrome (CSS) is a rare form of small vessel vasculitis characterized by asthma, hypereosinophilia and vasculitis affecting a variety of organs. We report a case of CSS presenting with visual loss due to posterior ischaemic optic neuropathy (PION), a previously unreported neurological complication of this form of vasculitis. A 60-yr-old male with a history of asthma and recurrent sinusitis presented to his general practitioner with 5 days of increasing shortness of breath, malaise and myalgia. An exacerbation of asthma secondary to a viral infection was felt to be the cause of his presentation and a short course of oral corticosteroids was prescribed that resulted in a marked improvement of his symptoms. Shortly after stopping corticosteroids his symptoms recurred; they were now associated with diplopia, jaw claudication, 6 kg weight loss and transient paraesthesias involving the left hand and foot. The patient was admitted to hospital for further investigation. On admission, the significant findings were diplopia with vertical separation of images, mild proximal muscle weakness, sensory peripheral neuropathy and widespread expiratory wheeze in the chest. Shortly after admission the patient developed acute painless loss of vision in his right eye. He was now found to have a right afferent pupillary defect, with a large central scotoma and visual acuity that was reduced to counting fingers. Fundoscopic examination revealed a normal disc with a swollen macula, findings consistent with the diagnosis of PION. Laboratory investigations showed a normal haemoglobin concentration and platelet count, a white cell count of 17x109/l with eosinophilia of 10.5x109/l (57%), normal renal function, creatine kinase of 6054 U/l (RR 0200), erythrocyte sedimentation rate 38 mm/h (RR 020) and a C-reactive protein of 127 mg/l (RR 05). Antinuclear antibodies were negative and antineutrophil cytoplasmic antibodies were positive, with a perinuclear pattern of staining on indirect immunofluorescence. Sinus radiography showed opacification of both the maxillary and the ethmoid sinuses. A temporal artery biopsy showed no evidence of large vessel vasculitis involving the temporal artery itself, but did demonstrate a prominent perivascular eosinophilic infiltrate and vascular inflammation involving smaller cutaneous vessels Fig. 1. A muscle biopsy revealed a mixed lymphocytic and monocytic interstitial inflammatory infiltrate consistent with a myositic process. A diagnosis of CSS was made and i.v. methylprednisolone 1 g daily was commenced. There was a rapid improvement in the patient's visual loss and resolution of his constitutional symptoms. The patient was discharged on oral corticosteroids and azathioprine, with visual acuity of 6/62 uncorrected in the affected eye.
In 1951 the pathologists Churg and Strauss reviewed the records of 13 patients with periarteritis nodosa who presented with a clinical syndrome characterized by asthma, hypereosinophilia and systemic vasculitis [1]. The three histological features common to these cases that were not present in periarteritis nodosa patients without asthma were extravascular granulomas, tissue eosinophilia and necrotizing vasculitis. Churg and Strauss coined the term allergic granulomatosis and angiitis to describe these 13 cases, which was later changed to CSS in recognition of their work in the classification of systemic vasculitis. Recognition that not all patients with CSS had the three pathological features originally described by Churg and Strauss led Lanham [2] to advocate a clinical approach to the diagnosis. Lanham et al. [2] proposed that the diagnosis of CSS could be made in patients with asthma, hypereosinophilia and clinical evidence of vasculitis involving two or more extrapulmonary organs. Another classification system states that the diagnosis of CSS can be made if at least four of the following six criteria are present: asthma, eosinophilia >10% of total white blood count, mono- or polyneuropathy, pulmonary infiltrates, sinus involvement and extravascular eosinophils in biopsy [3]. The unifying feature of patients presenting with CSS is asthma. Although wheeze is the most common reason for presentation, the vasculitic process is often outside the lungs, most commonly involving the peripheral nervous system, heart and gastrointestinal tract. Vasculitis involving the peripheral nervous system is the most characteristic feature of CSS, and mononeuritis multiplex occurs in 75% of patients. Other neurological manifestations of CSS described previously in association with CSS include peripheral neuropathy, cranial nerve palsies, stroke, dementia and anterior ischaemic optic neuropathy (ION) [4, 5]. The association of CSS and PION has not been described previously. Involvement of the eye is uncommon in CSS, but can include conjunctivitis, scleritis, uveitis, corneal ulceration and exophthalmos. Most reports of arteritic ION concern patients with temporal arteritis and are associated with permanent visual loss. Similar visual outcomes are seen in the CSS literature, with only one report of reversible visual loss due to PION. In a recently published retrospective study, however, Liu et al. [6] reported that over 30% of patients with visual loss secondary to arteritic ION experienced improvement of vision if treated with high-dose systemic corticosteroids early in the course. Our case report highlights two important aspects of vasculitis management: first, PION can be associated with CSS; secondly, the prompt institution of high-dose corticosteroids in patients with PION is important.
Notes
Correspondence to: T. T. Warner, University Department of Clinical Neurosciences, Royal Free and University College Medical School, Rowland Hill Street, London NW3 2PF, UK. 
References
- Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa. Am J Pathol1951;27:277301.[ISI]
- 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;63:6581.
- Masi AT, Hunder GG, Lie JT et al. The American College of Rheumatology 1990 criteria for the classification of ChurgStrauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum1990;33:94100.
- Alberts AR, Lasonde R, Ackerman KR, Chartash EK, Susin M, Furie RA. Reversible monocular blindness complicating Churg Strauss syndrome. J Rheumatol1994;21:3635.[ISI][Medline]
- Kattah JC, Chrousos GA, Katz PA, McCasland B, Kolsky MP. Anterior ischaemic optic neuropathy in ChurgStrauss syndrome. Neurology1994;44:22001.[Abstract]
- Liu GT, Glaser JS, Schatz NJ. Visual morbidity in giant cell arteritis. Ophthalmology1994;101:177985.[ISI][Medline]
Accepted 14 June 2000