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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2012  |  Volume : 23  |  Issue : 2  |  Page : 330-333
A case of exorbitism in association with Wegener's granulomatosis with renal involvement

1 Department of Nephrology, La Rabta Hospital, Tunis, Tunisia
2 Ophtalmology Institute, Hedi Rais, Tunis, Tunisia
3 Laboratoire de Recherche de Pathologie, Rénale LR00SP01, Tunisia

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Date of Web Publication28-Feb-2012


Wegener's granulomatosis (WG) is a necrotizing granulomatous vasculitis invol­ving the nose, paranasal sinuses, lungs, and kidneys. Ocular involvement can occur in about 50% of cases. There are very few reports of WG with orbital inflammation and exorbitism. We report a case of a female patient who presented with exorbitism related to orbital inflammation secondary to WG, with renal involvement. A 29-year-old woman with a previous history of recurrent pan-sinusitis presented with bilateral exophthalmos and renal failure with rapidly progressive glo-merulonephritis. Computed tomography showed extensive bilateral soft tissue in the retro-orbital area. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies and renal biopsy revealed pauci immune crescentic glomerulonephritis. The patient was treated with corticosteroids and pulses of cyclophosphamide followed by azathioprine and trimethoprim-sulfamethoxazole. After a follow-up of 10 months, the renal outcome was favorable with improvement of renal function but there was persistence of exorbitism and loss of visual function. Our case suggests that WG should be considered in the differential diagnosis of persistent bila­teral exophthalmos. Prompt recognition of this early manifestation is important for the institution of early treatment.

How to cite this article:
Beji S, Fatma L B, Chebbi A, Rais L, Krid M, Smaoui W, Maiz H B, Zouaghi K, Moussa F B. A case of exorbitism in association with Wegener's granulomatosis with renal involvement. Saudi J Kidney Dis Transpl 2012;23:330-3

How to cite this URL:
Beji S, Fatma L B, Chebbi A, Rais L, Krid M, Smaoui W, Maiz H B, Zouaghi K, Moussa F B. A case of exorbitism in association with Wegener's granulomatosis with renal involvement. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2022 Jun 25];23:330-3. Available from: https://www.sjkdt.org/text.asp?2012/23/2/330/93168

   Introduction Top

Wegener's granulomatosis (WG) is a necro-tizing granulomatous vasculitis involving the nose, paranasal sinuses, lungs, and kidneys. It is characterized by a classic triad of granulo-matous inflammation of the respiratory tract, necrotizing vasculitis, and nephritis. The diag­nosis is based on clinical criteria, presence of anti-neutrophil cytoplasmic antibodies (ANCA), and signs of granulomatous necrotizing vascu-litis on histology. Approximately 50% of pa­tients with WG develop ophthalmic disease. There are very few reports on WG presenting as exophthalmos. We report a case of a female patient who pre­sented with exorbitism related to orbital inflammation in association with WG, with renal involvement

   Case Report Top

A 29-year-old woman with a nine month history of pan-sinusitis, treated with antibio­tics, presented with progressive bilateral exoph-thalmos and acute renal failure. Clinical exa­mination revealed blood pressure of 120/70 mmHg, absence of edema, right exophthalmos and proptosis, and 2+ proteinuria and 3+ he-maturia on urinalysis [Figure 1]. Laboratory tests revealed the following: proteinuria of 0.7 g/day, serum total protein of 72 g/L, serum albumin of 36 g/L, gamma globulin of 16 g/L, serum creatinine of 7.2 mg/dL, hemoglobin of 8 g/dL, total leukocyte count of 12,500/mm [3] , platelets of 210,000/mm [3] , erythrocyte sedi­mentation rate of 120 mm/hour and C-reactive protein of 52 mg/dL. Liver function tests were normal. Chest radiograph and renal ultrasound were normal. Computed tomography (CT) showed extensive bilateral soft tissue in the sinus with extension to the retro-orbital area [Figure 2]. The association of pan-sinusitis, bilateral orbital inflammation, and rapidly pro­gressive glomerulonephritis suggested a diag­nosis of vasculitis. Immunologic tests showed the presence of c-ANCA and anti-proteinase-3 (PR-3) antibody. Renal biopsy was performed which revealed pauci immune crescentic glo-merulonephritis. The patient was treated with three pulses of methyl prednisolone followed by oral corticosteroids at a dose of 1 mg/kg/ day for two months with progressive tapering and six monthly pulses of cyclophosphamide at a dose of 750 mg/1.73 m [2] /pulse followed by azathioprine at a dose of 2 mg/kg/day and tri-methoprimsulfamethoxazole. After a follow-up of ten months, the renal outcome was favo­rable with improvement of renal function (serum creatinine 1.6 mg/dL). However, proptosis and loss of vision persisted.
Figure 1: Clinical photograph showing features of right exophthalmos and left proptosis.

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Figure 2: Computed tomography scan showing bilateral extensive soft tissue in the sinus with extension to the retro-orbital area.

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   Discussion Top

WG can affect almost any organ system. It often presents with nonspecific symptoms and signs, and hence may be misdiagnosed. Oph­thalmic involvement is relatively common in the course of the disease with a reported inci­dence varying among different series, but it is usually estimated to occur in 50-60% of patients. [1],[2]

Ocular involvement in WG is multiple and may result in conjunctivitis, episcleritis, kera-titis, scleritis, uveitis as well as vasculitis of the retina. Ocular involvement may also be in the form of proptosis, optic neuritis, nasolac-rimal duct obstruction, altered motility, and orbital inflammation. [3] Orbital involvement can be seen in 15-40% and may be an extension of the sinus inflammation or an orbital pseu-dotumor independent of the sinus disease. [4]

The most common presentation of orbital disease involves a subacute prodromal phase with escalating pain and proptosis evolving over one to two months, followed by a brief acceleration phase when optic nerve compres­sion and loss of vision occur. This is often bilateral and usually causes irreversible mor­bidity if it is not treated. There are few cases in literature of WG presenting with proptosis secondary to orbital inflammation. [5],[6]

The features on CT scan which are sugges­tive of a diagnosis of orbital WG include involvement of the sinus structures and an or­bital mass. There is often obliteration and infil­tration of adjacent fat planes that may include osseous erosions. [7] CT scan can also demons­trate the irregular, ulcerated surface of the lining granulomatous tissue. More recently, the use of magnetic resonance imaging (MRI) as a diagnostic tool has been highlighted. [8] Al­though it may not be fully validated for vas-culitis, MRI can detect granulomas and better delineate mucosal inflammation and ulceration in the sinuses, nasal cavity, and the orbits.

Ocular involvement may be the initial pre­senting feature of WG in some patients. It is therefore imperative to consider WG in the differential diagnosis. Thyroid or Graves' orbi-topathy is the most common cause of orbital inflammatory disease and should always be considered in all patients who present with orbital inflammation or an orbital mass. [9] Cha­racteristic ocularmanifestations, such as eyelid retraction, along with other systemic signs and symptoms of thyroid disease, as well as appro­priate investigations for thyroid dysfunction will help in diagnosis.

Idiopathic orbital inflammation, also known as orbital pseudotumor, is a clinical syndrome characterized by a nonspecific pattern of chro­nic inflammation manifesting as an orbital mass. [10] It frequently presents with an abrupt onset of proptosis, pain, and ocular inflam­matory symptoms and signs, without an iden­tifiable local or systemic cause. It is a diag­nosis of exclusion relying on information ob­tained from history, physical examination, imaging, and biopsy.

The presence of bilateral orbital inflamma­tion, involvement of the upper respiratory tract and sinuses, presence of microscopic hema-turia and rapidly progressive renal failure raised the suspicion of WG in our patient. Diagnosis of WG was confirmed by the pre­sence of ANCA as well as biopsy. Orbital biopsy can be obtained through an endoscopic transnasal/trans-ethmoidal approach, external ethmoidectomy, or orbitotomy. [11] In our patient, orbital biopsy was not performed as renal biopsy showed features of pauci immune ex-tracapillary glomerulonephritis with vasculitis.

Testing for ANCA, by using either immuno-fluorescence (IF) and/or enzyme-linked immu-nospecific assay (ELISA), is of significant value in the diagnosis of certain vasculitides. Using ELISA, the c-ANCA pattern reacts with PR-3, whereas the p-ANCA pattern typically reacts with myeloperoxidase (MPO). [12] The c-ANCA is associated with a sen-sitivity of 91% and a specificity of 99% for WG. [13] On the other hand, the sensitivity of p-ANCA/anti-MPO for WG is reported to be around 10-12%, and hence it is not considered as a reliable marker.

In all cases of WG, treatment should be instituted early. Involvement of the respiratory and renal systems may be life threatening without treatment. These patients may present initially with ocular involvement, as seen in our patient. Treatment of ANCA-associated vascu-litis is now well defined, but must be adjusted for each patient according to the type of vas-culitis, its precise form and severity, and pa­tient's characteristics such as age and renal function. The therapeutic decision must also take into account the risk of adverse events inherent to each treatment. The efficacy of adequate induction treatment has been de­monstrated and more than 80% of patients now achieve remission. [14] Relapse rates none­theless remain high, especially in WG. Patients with WG must receive a combination of cor-ticosteroids and immunosuppressive agents, mainly intravenous pulse cyclophosphamide. The optimal duration of induction therapy re­mains to be determined, but should not be shorter than 18 months. Plasma exchange is indicated as an adjuvant therapy for patients with severe renal involvement. Once remission is achieved, maintenance therapy can be with a less toxic immunosuppressive drug such as azathioprine or methotrexate in place of cyclo-phosphamide. [15] In our patient, combination of corticosteroids and pulse cyclophosphamide was initiated because of renal, ocular, and sinus involvement.

Although aggressive medical therapy can improve orbital symptoms, proptosis associated with orbital WG can be refractory to conven­tional treatment. [16] The deterioration of ocular inflammation in our patient with otherwise well-controlled disease may indicate delay in diagnosis and treatment.

Our case suggests that WG should be consi­dered in the differential diagnosis in patients who present with persistent signs of bilateral exophthalmos and pan-sinusitis. Prompt recog­nition of this manifestation is of at most im­portance for the institution of early treatment, thereby avoiding serious complications.

   References Top

1.Pakrou N, Selva D, Leibovitch I. Wegener's Granulomatosis: Ophthalmic manifestations and ma­nagement. Semin Arthritis Rheum 2006;35(5):284-92.   Back to cited text no. 1
2.Bullen CL, Liesegang TJ, McDonald TJ, De Remee RA. Ocular complications of Wegener's granulo-matosis. Q J Med 1993;90:279-90.  Back to cited text no. 2
3.Thorne JE, Jabs DA. Ocular manifestations of vas-culitis. Rheum Dis Clin North Am 2001;27:761-79.  Back to cited text no. 3
4.Sadiq SA, Jenning CR, Jones NS, Downes RN. Wegner's granulomatosis: the ocular manifestations revisited. Orbit 2000;19:253-61.  Back to cited text no. 4
5.Perry SR, Rootman J, White VA. The clinical and pathologic constellation of Wegener granulomatosis of the orbit. Ophthalmology 1997;104(4):683-94.  Back to cited text no. 5
6.Fechner FP, Faquin WC, Pilch BZ. Wegener's granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope 2002;112:1945-50.  Back to cited text no. 6
7.Provenzale JM, Mukherji S, Allen NB, Castillo M, Weber AW. Orbital involvement by Wegener's granulomatosis: imaging findings. AJR Am J Roentgenol 1996;166(4):929-34.  Back to cited text no. 7
8.Silvera S, Vignaux O, Legmann P. Sinonasal and cerebral imaging findings in Wegener's granulo-matosis. Presse Med 2007;36:913-21.  Back to cited text no. 8
9.Bradley EA. Graves ophthalmopathy. Ophthalmology 2001;12:347-9.  Back to cited text no. 9
10.Ahn Yuen SJ, Rubin PAD. Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome. Arch Ophthalmol 2003;121:491-9.  Back to cited text no. 10
11.Kalina PH, Lie JT, Campbell RJ, Garrity JA. Diag­nostic value and limitations of orbital biopsy in Wegener's granulomatosis. Ophthalmology 1992;99: 120-4.  Back to cited text no. 11
12.Savige J, Pollock W, Trevisin M. What do anti-neutrophil cytoplasmic antibodies (ANCA) tell us? Best Pract Res Clin Rheumatol. 2005;19(2):263-76.  Back to cited text no. 12
13.Rao JK, Weinberger M, Oddone EZ, Allen NB, Landsman P, Feussner JR. The role of anti-neutrophil cytoplasmic antibody (c-ANCA) testing in the diagnosis of Wegener granulomatosis. A literature review and meta-analysis. Ann Intern Med 1995;123:925-32.  Back to cited text no. 13
14.Chen M, Yu F, Zhao MH. Relapses in patients with antineutrophil cytoplasmic autoantibody-associated vasculitis: likely to begin with the same organ as initial onset. J Rheumatol 2008; 35(3):448-50.  Back to cited text no. 14
15.Guillevin L, Pagnoux C. Treatment of ANCA-asso-ciated vascularitidis. Presse Med 2007; 36:922-7.  Back to cited text no. 15
16.Bhatia A, Yadava U, Goyal JL, Chaturvedi KU. Limited Wegener's granulomatosis of the orbit: a case study and review of literature. Eye 2005;19 (1):102-4.  Back to cited text no. 16

Correspondence Address:
S Beji
Department of Nephrology, La Rabta Hospital, Jabbari 1007, Tunis
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Source of Support: None, Conflict of Interest: None

PMID: 22382229

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