Wegener's granulomatosis as a cause of cicatrising conjunctivitis
- Wolfgang Bernauer, MD, OMMA Eye Center, Theaterstrasse 2, 8001 Zurich, Switzerland
- Accepted 15 November 2000
Editor,—Wegener's granulomatosis is a multisystem disorder characterised by the classic triad of necrotising granulomas in the upper respiratory tract and the lung, a variable degree of systemic small vessel vasculitis, and a focal necrotising glomerulonephritis.1 A limited form of Wegener's granulomatosis, with absence of glomerulonephritis, has been described.2
Ophthalmic complications occur in about 30% of patients with biopsy proved disease.3 Among these orbital pseudotumours ulcerations of the sclera and the cornea are observed most frequently and the histopathological findings have been described.3Involvement of the conjunctiva and eyelids has also been observed,4 but progressive scarring of the conjunctiva and its sequelae has not been reported.
A 72 year old man was first seen at our department in November 1997 with bilateral conjunctivitis. His medical history had been unrevealing until early 1987, when he developed systemic illness with upper respiratory complaints and renal insufficiency. Wegener's granulomatosis was diagnosed by biopsy of the nasal mucosa and kidney. In 1993 he developed necrotising anterior scleritis and peripheral corneal melting in the left eye. There was complete remission on systemic immunosuppressive therapy.
Since January 1997 the patient has suffered from recurrent bilateral conjunctivitis. He complained about persistent epiphora in the left eye and irritation in both eyes. He was then on local treatment with fusidic acid gel and prednisolone 1% eye drops for 2 weeks. On examination lid margin disease with obstruction of the meibomian glands and obliteration of the left canaliculi was noted. The left upper lid showed inflammatory thickening with haemorrhagic and mild cicatricial changes of the tarsal conjunctiva (Fig 1A). In the right eye similar changes were noted; they were, however, much less prominent. Bulbar conjunctiva was unremarkable and there were no other signs of active inflammation in the globe. As there was at that time no evidence of systemically active Wegener's granulomatosis and as cANCA levels were near normal, systemic immunosuppressive treatment was not started again and therapy was restricted to the usual blepharitis regimen (oral tetracycline, lubricating agents, local antibiotics, and steroids).
The patient was referred again in March 1999 after he had suffered several episodes of superficial corneal ulceration in the left eye. On examination the left upper lid was less inflamed than 2 years earlier, but progressive scarring of the tarsus with cicatricial entropion and trichiasis had occurred (Fig 1B). The right upper eyelid showed marked inflammatory thickening, clinically imposing as multiple chalazia. A biopsy from the lateral right upper tarsus disclosed a chronic infiltration with lymphocytes, plasma cells, and occasional histiocytes. Numerous eosinophils but no giant cells were noted. Perivascular inflammation (Fig 2) and areas of active necrosis were only seen in few areas while disorganisation of the tarsus by fibrous tissue was obvious. Together with the analysis of extraocular tissue and the clinical background, the conjunctival biopsy was regarded as diagnostic.
The current state of knowledge has not recognised Wegener's granulomatosis as a disorder causing cicatrising conjunctivitis.5 The diagnosis of granulomatous conjunctivitis in Wegener's granulomatosis can easily be established in the presence of a history of systemic disease. Conjunctival and eyelid findings, however, may be the presenting symptoms4and correct diagnosis is difficult. cANCA levels, a chestx ray film, and an urinary sediment may be helpful in such situations.
Owing to the small number of cases, the experience in treating conjunctival Wegener's disease is limited. In the absence of detectable cANCA levels or extraocular disease activity, we did initially not recommend systemic treatment with steroids and/or immunosuppressive agents. Considering the progressive course with severe lid complications this approach has to be redefined. Further reports will help in this decision.