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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 897-899

Anterior nodular scleritis – tuberculosis or granulomatosis with polyangiitis? A series of three cases


1 Deparment of Ophthalmology, Kalinga Institute of Medical Sciences; Department of Ophthalmology, Vision Care, Centre for Retina, Bhubaneswar, Odisha, India
2 Department of Ophthalmology, Uvea, Sankara Nethralaya, Kolkata, West Bengal, India
3 Deparment of Obstetrics and Gynaecology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India
4 Deparment of Clinical Immunology and Rheumatology, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India
5 Department of Ophthalmology, Vision Care, Centre for Retina, Bhubaneswar, Odisha, India

Date of Submission23-Mar-2022
Date of Acceptance05-Aug-2022
Date of Web Publication11-Oct-2022

Correspondence Address:
Dr. Nidhi Sinha
M-1, Samanta Vihar, Near NALCO Chowk, Bhubaneswar - 751 017, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_751_22

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  Abstract 


Scleritis is a chronic destructive inflammatory disorder. It has various systemic associations, including granulomatosis with polyangiitis (GPA). However, anterior nodular scleritis being the initial manifestation of GPA is rare. Being in an endemic country of tuberculosis (TB), it is imperative to exclude any possibility of active or latent TB before institution of immunosuppressive agents. Similarly, the vice versa should be kept in mind. We present three cases of persistent anterior nodular scleritis even after treatment with antitubercular treatment (ATT). Subsequent recurrences led to the evaluation and diagnosis of GPA with multisystem involvement. Treatment with immunosuppressive therapy led to successful recovery.

Keywords: AntiNeutrophil Cytoplasmic Antibodies, antitubercular treatment (ATT), granulomatosis with polyangiitis (GPA), immunosuppressive therapy, scleritis


How to cite this article:
Rao G N, Patnaik G, Prasanna G, Padhan P, Sinha N. Anterior nodular scleritis – tuberculosis or granulomatosis with polyangiitis? A series of three cases. Indian J Ophthalmol Case Rep 2022;2:897-9

How to cite this URL:
Rao G N, Patnaik G, Prasanna G, Padhan P, Sinha N. Anterior nodular scleritis – tuberculosis or granulomatosis with polyangiitis? A series of three cases. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Nov 27];2:897-9. Available from: https://www.ijoreports.in/text.asp?2022/2/4/897/358188



Anterior nodular scleritis has relatively lower systemic association when compared to other subtypes of scleritis, especially necrotizing scleritis,[1] although in a study of 140 eyes with anterior nodular scleritis, 22% had it.[2] Associated tuberculosis (TB) and granulomatosis with polyangiitis (GPA) both are reported. In India, being an endemic country for TB, it is diagnosed early and sometimes leads to overdiagnosis and inappropriate use of antitubercular treatment (ATT). The cases presented here were initially being treated for TB solely based on immunological tests. Subsequent recurrences led to the evaluation and diagnosis of GPA with multisystemic involvement.


  Case Series Top


Case 1

A 36-year-old female presented with headache, redness, pain, and 6/9 vision in left eye. She was treated elsewhere for episcleritis, but persisting symptoms led to investigations that showed raised erythrocyte sedimentation rate (ESR; 56 mm in the first hour), positive Mantoux test (20 mm induration), and bilateral hyperdense foci with perilesional ground glass opacities on contrast-enhanced computerized tomography (CECT) thorax. Hence, she was started on ATT along with oral prednisolone 1 mg/kg/day, but she had no relief. After 6 months, she presented to us with severe pain and persisting redness. On examination, edematous and congested scleral vessels were noticed [Figure 1]a and [Figure 1]b. Fundus examination was unremarkable. She denied past history of systemic illness or any similar family history. Along with history of fever, weight loss, headache, and cough, she had pansinusitis in Computed Tomography Para Nasal Sinuses (CT PNS) and urine examination showed microscopic hematuria. She had raised serum cytoplasmic Anti Neutrophil Cytoplasmic Antibodies (c- ANCA) level (35.82 AU/ml, normal <2 AU/ml). She was diagnosed as a case of GPA based on upper airways, lower airways, and renal involvement along with raised c-ANCA levels.
Figure 1: Slit-lamp images of Case 1 showing (a) anterior nodular scleritis at presentation, (b) scleral congestion and edema at presentation, and (c) resolved scleritis after treatment

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She received intravenous (IV) pulse methyl prednisolone 500 mg once daily for 3 days with inj. rituximab 1000 mg every 2 weeks for two doses as an induction therapy followed by 500 mg every 6 months as maintenance with tapering doses of steroids.[3] At 6 months follow-up, she had no pain, quiet eye with resolved scleritis, and maintained 6/9 vision in that eye [Figure 1]c.

Case 2

A 48-year-old female presented with recurrent painful red eye and 6/6 vision in right eye. She was diagnosed elsewhere with anterior nodular scleritis, and ATT was started due to positive Mantoux test and raised ESR, but they gave her no relief in symptoms. On examination, a nodular scleritis was seen [Figure 2]a and [Figure 2]b. Fundus examination was unremarkable. She gave a history of fever, cough, weight loss, hemoptysis, and recurrent otitis media for 1 year. CT scan of the mastoid region confirmed presence of chronic mastoiditis. She had proteinuria and microscopic hematuria, and CT scan of chest showed multiple lung nodules. Her c-ANCA was positive (7.16 AU/ml, normal <2 AU/ml). She was diagnosed as GPA.
Figure 2: Slit-lamp images of Case 2 showing (a) anterior nodular scleritis at presentation, (b) scleral congestion at presentation, and (c) resolved scleritis after treatment

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She was started on IV pulse methyl prednisolone 500 mg once daily for 3 days followed by oral prednisolone 1 mg/kg/day along with azathioprine 2 mg/kg/day as steroid-sparing agents.[3] Within 6 months, steroids could be tapered to 7.5 mg/day along with azathioprine 100 mg/day with no relapse [Figure 2]c.

Case 3

A 50-year-old female presented to us with redness, pain, and 6/24 vision in her right eye. On examination, anterior nodular scleritis was seen [Figure 3]a. Fundus examination was unremarkable. She had cough, hemoptysis, and occasional shortness of breath and was diagnosed with pulmonary TB elsewhere. She was receiving ATT for the same. She gave a history of similar episode of redness and pain in the same eye 1 month back. On evaluation, she had fever, weight loss, epistaxis, sinusitis, hypertension, hematuria and proteinuria along with multiple lung nodules in chest radiograph. Her c-ANCA was elevated (37.35 AU/ml, normal <2 AU/ml), and she was diagnosed to have GPA.
Figure 3: Slit-lamp image of Case 3 showing (a) anterior nodular scleritis at presentation, (b) relapse after 6 months, and (c) resolved scleritis with scleral thinning after treatment

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She received IV pulse methyl prednisolone 500 mg once daily for 3 days followed by oral prednisolone 1 mg/kg/day along with IV pulse cyclophosphamide as per the European Vasculitis Study Group (EUVAS) protocol. However, due to relapse after 6 months [Figure 3]b, she received inj. rituximab 500 mg every week for four doses followed by 500 mg every 6 months as maintenance therapy. She maintained 6/24 vision in that eye on subsequent visits with no further relapses [Figure 3]c.[3]


  Discussion Top


The cases presented here had diseases mimicking TB, but detailed evaluation led to diagnosis of GPA. The cases were diagnosed as GPA based on the American College of Rheumatology (ACR) criteria.[4] It is known that GPA can provoke nodular, diffuse, or necrotizing scleritis, which can be severe than scleritis of other etiologies.[5] But bilateral diffuse or necrotizing anterior scleritis with corneal involvement is the more common presentation in GPA.[1] All our patients presented with unilateral, anterior nodular scleritis.

The similarities between TB and GPA are fever, weight loss, cough, hemoptysis, and lung findings in imaging. Cross reactivity between ANCA and the mycobacterial phenolic glycolipids complex is also known. In a study of 45 known TB cases, ANCA was found positive in 44.4% cases.[6] But positive tuberculin test also has a poor positive predictive value for current disease.[7] Our patients, although tuberculin test positive, showed no improvement on receiving ATT elsewhere. Cases of overdiagnosis of TB in limited GPA patients with lung and ear involvement have been reported before.[8],[9]

ANCA immunofluorescence (IF) qualitative assays identify cytoplasmic ANCA, Perinuclear - AntiNeutrophil Cytoplasmic Antibodies (p-ANCA), and atypical ANCA. Enzyme-linked immunosorbent assays (ELISA) are quantitative and measure Proteinase 3 ANCA (PR3-ANCA) and Myelperoxidase ANCA (MPO-ANCA) titers. The use of IF and ELISA in ANCA testing gives a 96% sensitivity and 98.5% specificity for ANCA-associated vasculitis, with 88% GPA patients being seropositive for c-ANCA.[4]


  Conclusion Top


The diagnosis of anterior nodular scleritis can be challenging. In chronic and recurrent scleritis, one should evaluate for systemic autoimmune diseases like vasculitis as in our series.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Dutta Majumder P, Sudharshan S, George AE, Ganesh SK, Biswas J. Antineutrophil cytoplasmic antibody-positive scleritis: Clinical profile of patients from a tuberculosis-endemic region. Indian J Ophthalmol 2018;66:1587-91.  Back to cited text no. 1
    
2.
Patnaik G, Sudharshan S, George AE, Ganesh SK, Biswas J, Dutta Majumder P. Clinical profile of patients with anterior nodular scleritis in India. Indian J Ophthalmol 2020;68:1925-8.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Samman KN, Ross C, Pagnoux C, Makhzoum J-P. Update in the management of ANCA-associated vasculitis: Recent developments and future perspectives. Int J Rheumatol 2021;2021:5534851.  Back to cited text no. 3
    
4.
Lutalo PM, D'Cruz DP. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis). J Autoimmun 2014;48:94-8.  Back to cited text no. 4
    
5.
Sfiniadaki E, Tsiara I, Theodossiadis P, Chatziralli I. Ocular manifestations of granulomatosis with polyangiitis: A review of the literature. Ophthalmol Ther 2019;8:227-34.  Back to cited text no. 5
    
6.
Flores-Suarez L. Prevalence of antineutrophil cytoplasmic autoantibodies in patients with tuberculosis. Rheumatology (Oxford) 2003;42:223-9.  Back to cited text no. 6
    
7.
Nayak S, Acharjya B. Mantoux test and its interpretation. Indian Dermatol Online J 2012;3:2-6.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Laushkina Z, Pushkareva E, Medvedev S, Filimonov P, Yagubkin P. Granulomatosis with polyangiitis (Wegener's): A clinical case of overdiagnosis of pulmonary tuberculosis. Pulmonologiya 2022;32:130-6.  Back to cited text no. 8
    
9.
Breuskin F, Polet M, Dorzee J, Fastrez J, Van Eeckhout P, Henkinbrant A, et al. Limited Wegener's disease initially misdiagnosed as tuberculosis. Acta Clinica Belg 2003;58:245-7.  Back to cited text no. 9
    


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