Orbital Wegener's Granulomatosis Research Articles

Authors' response to Meredith and Hull

We thank Meredith and Hull for the interest in our article about orbital Wegener's granulomatosis in a 9-year-old child as the initial presentation.1 As stated in the article it is important for these patients to be managed by specialist centres which deal with paediatric immunological disease.

Response to ‘Orbital Wegener's granulomatosis as the initial presentation in a 9-year-old child’

We were interested to read the article by Dey et al. describing the ocular presentation of Wegener's granulomatosis in a child.1 The authors considered corticosteroids to be the first line of treatment in this condition. This varies from the usual induction regimen, which is a combination of cyclophosphamide and corticosteroid. Wegener's granulomatosis, often referred to as granulomatosis with polyangiitis, is a fatal disease, with mortality if untreated of 90% at two years. In 1973, Fauci and Wolff showed that a regimen that combined cyclophosphamide and prednisolone induced disease remission.2 This combination was superior to corticosteroid treatment alone. Subsequent long-term experience has demonstrated that the cyclophosphamide and prednisolone regimen induces remission in 85–90% of patients within two to six months.3 More recently the introduction of the anti B-cell monoclonal antibody, rituximab, has provided an alternative induction therapy to cyclophosphamide or for use in cyclophosphamide-resistant disease.4 It is currently recommended as an initial induction regimen only in those patients who have a contraindication to cyclophosphamide. While single therapy with either corticosteroids or co-trimoxazole have had their advocates, current advice is that in treatment of limited disease combination therapy with corticosteroid and either methotrexate or cyclophosphamide should be used. Co-trimoxazole can be used as part of maintenance therapy to prevent Pneumocystis infection.5 Once remission is achieved patients are then switched to maintenance immunosuppression such as methotrexate or azathioprine. Thus corticosteroids alone appear inferior for both induction and maintenance therapy.

Orbital Wegener's granulomatosis as the initial presentation in a 9-year-old child

A 9-year-old child was referred by her general practioner (GP) to eye casualty clinic with a six-week history of unexplained painless swelling of her right upper lid. Her GP, having treated first as an allergy with oral antihistamine, was now concerned that this was a case of preseptal cellulits progressing to orbital cellulitis and commenced a course of Erythromycin and Fucithalmic ointment and referred for review. A detailed history had revealed four months previously she had developed right upper lid swelling in the context of a mild upper respiratory tract infection. However this was short-lived and resolved without any therapeutic intervention. She also frequently suffered from recurrent ear infections, which required eventual grommet insertion, and nose bleeds. On examination she was found to have right painless upper lid swelling with no associated proptosis (Figure 1). Her ocular movements were full with no diplopia. Best corrected vision was 6/9 both eyes, her conjunctiva was injected in the right eye, anterior segment examination was normal with pressures of 14 both eyes. Pupils were reactive and equal with, no evidence of a relative afferent pupillary defect. Colour vision was also full. Posterior segment examination was normal no evidence of disc swelling or pallor. The antibiotics were continued with a provisional diagnosis of dacroadenitis. In view of the chronicity of the symptoms an orbital computed tomography scan and bloods were ordered. Her CT scan displayed diffuse homogenous soft tissue swelling within the right lacrimal gland suggesting an inflammatory process, extraocular eye muscles were normal (Figure 2). Her chest X-ray was normal and urine analysis was also normal. Her blood tests was positive for cANCAwith a titre of 1:20 and pANCAwith a titre of 1:40, ESR was normal at 11 the rest of her blood tests including full blood count, U&Es, LFTs, autoantibody screen and sACE were also normal. She was referred on to a paediatric rheumatologist to confirm the diagnosis of Wegener’s granulomatosis and assist on further management. A biopsy of the lacrimal failed to show the classical triad of granulomatous inflammation with giant cell, necrosis and vasculitis although there was evidence of chronic inflammation with perivascular infiltrates. Further examination of the child revealed saddling of the nose and a congenital sinus in the tragus of the left ear. Systemic examination failed to reveal any renal or pulmonary involvement. In view of the positive ANCA and clinical examination, a diagnosis of Wegener’s granulomatosis was made. She was commenced initially with intravenous methylprednisolone for three days then on a tapering dose of oral steroids. The lid swelling has reduced and she is currently in remission.

Wegener's granulomatosis of the orbit: two cases requiring endoscopic surgical decompression

We report two unusual and contrasting cases of Wegener's granulomatosis involving the orbit, requiring urgent endoscopic orbital decompression surgery. Both patients presented with an acute deterioration in visual function which failed to improve with medical management. Case one presented with systemic features of the condition, in contrast with case two, who presented with a more localised form of the disease. Whereas case one exhibited primary orbital disease with maxillary sinus extension (i.e. focal disease), case two illustrates orbital involvement secondary to sinus disease extension (i.e. contiguous disease). Prompt diagnosis, assisted by the presence of systemic features, led to a good visual outcome in case one. In case two, in which diagnosis was difficult and surgery delayed, the outcome was poor. These cases of orbital Wegener's granulomatosis illustrate the diagnostic challenge, the requirement for early intervention following acute visual deterioration and the importance of heightened awareness of the rarer ENT manifestations of this disease.

Superior Oblique Myositis as an Early Feature of Orbital Wegener's Granulomatosis

A 66-year-old female presented with a 6-month history of increasing right-sided facial pain and diplopia. Right ocular movements were restricted in adduction and downgaze. Superior oblique (SO) enlargement was seen on an initial MRI scan, and subsequent diffuse infiltration was seen on a repeat scan. An elevated cANCA and biopsy of SO confirmed Wegener's granulomatosis (WG). We report a case of SO myositis as a predominant early feature of WG granulomatosis and review the literature regarding extraocular muscle involvement in WG. To our knowledge, early SO involvement has not been reported.

Antithymocyte globulin treatment of orbital Wegener granulomatosis: a follow-up study

PURPOSE: To describe the follow-up of patients with orbital Wegener granulomatosis after antithymocyte globulin treatment. METHODS: Patients with ocular/orbital Wegener granulomatosis refractory to standard treatment were selected for immunotherapy with rabbit antithymocyte globulin intravenously. The specific ocular/orbital symptoms were monitored in patients with a vision-threatening form of Wegener granulomatosis or a life-threatening form with ocular symptoms. RESULTS: One patient had a complete remission, two patients had remissions but still needed additional treatment, and one patient remained refractory. CONCLUSIONS: In selected patients, antithymocyte globulin immunotherapy may present an alternative when vision or life is threatened by orbital Wegener granulomatosis.