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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2021  |  Volume : 32  |  Issue : 6  |  Page : 1790-1794
Recurrent Scleritis and Immunoglobulin A Nephropathy - A Case Series and Literature Review of an Unusual Association


Department of Nephrology, NU Hospitals, Bengaluru, Karnataka, India

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Date of Web Publication27-Jul-2022
 

   Abstract 


Immunoglobulin A (IgA) nephropathy is usually restricted to the kidneys in most cases, but associations with other immune and inflammatory diseases exist. Scleritis, however, is an unusual association. We present an observational case series of two patients who initially presented with recurrent episodes of scleritis. A thorough evaluation for recurrent scleritis did not reveal any secondary cause per se. They were further evaluated extensively for incidental proteinuria and microscopic hematuria. Renal function was normal. Renal biopsy was performed which revealed IgA nephropathy in both the patients. They were given oral prednisolone and telmisartan for six months and followed for nine and six months, respectively, after steroids were discontinued. Proteinuria remitted, renal function remained normal, and there were no further episodes of scleritis in these patients.

How to cite this article:
Farid MS, Nayak NM, Modi S, Ramakrishnan S, Rangarajan D, Subramanian P. Recurrent Scleritis and Immunoglobulin A Nephropathy - A Case Series and Literature Review of an Unusual Association. Saudi J Kidney Dis Transpl 2021;32:1790-4

How to cite this URL:
Farid MS, Nayak NM, Modi S, Ramakrishnan S, Rangarajan D, Subramanian P. Recurrent Scleritis and Immunoglobulin A Nephropathy - A Case Series and Literature Review of an Unusual Association. Saudi J Kidney Dis Transpl [serial online] 2021 [cited 2022 Aug 15];32:1790-4. Available from: https://www.sjkdt.org/text.asp?2021/32/6/1790/352442



   Introduction Top


Immunoglobulin A (IgA) nephropathy, also known as Berger’s disease, remains one of the most common forms of glomerulonephritis.[1] It is characterized by deposition predominantly of IgA in the mesangium with mesangial proliferation. The clinical features span the spectrum from asymptomatic hematuria to rapidly progressive glomerulonephritis. Although, previously considered a benign disease, it is now evident that up to 40% of patients may progress to end-stage kidney disease,[2] thus necessitating early diagnosis. IgA nephropathy is also associated with and may be secondary to a variety of disease processes, particularly immune and inflammatory disorders.[2]

Ocular involvement in patients with IgA nephropathy is rare, and the commonly reported associations are with uveitis. Moreover, scleritis is not well known to occur in IgA nephropathy. Only few have reported its association with IgA nephropathy.[3],[4],[5],[6],[7] Scleritis may occur as an isolated phenomenon or as a manifestation of a variety of rheumatological diseases, infections, and metabolic disorders.

Here, we present an observational case series of two patients who initially presented with recurrent episodes of scleritis and were subsequently diagnosed to have underlying IgA nephropathy on evaluation for incidental proteinuria and microscopic hematuria.


   Case Reports Top


Case 1

A 35-year old female presented with repeated painful red eye episodes and was diagnosed to have recurrent anterior scleritis by her ophthalmologist. She had four episodes in a span of 18 months, with only one eye being affected. There was no vision disturbance and no history of trauma or ocular surgery. She did not have a prior history of connective tissue disease or any associated symptoms such as fever, rash, joint pains, excessive hair loss, photosensitivity, loss of appetite, lymphadenopathy, or genital ulceration at presentation. She had received topical steroids and symptomatic treatment for her initial scleritis episodes. Considering primary scleritis, she was also treated with methotrexate by a rheumatologist for a short duration. Her clinical examination was normal. She did not have hypertension (HTN) [Table 1]. Evaluation for common secondary causes of scleritis was negative. She had normal hemoglobin and blood counts. Erythrocyte sedimentation rate was mildly elevated. Rheumatoid factor and antinuclear antibody test were negative. Serum ACE level done to look for sarcoidosis was normal. Serology for human immunodeficiency virus, hepatitis B, hepatitis C, and syphilis was also negative. Chest X-ray was normal. However, her urinalysis revealed subnephrotic proteinuria with active sediments. She had normal renal function. Liver function tests were normal too. Serology for vasculitis (antineutrophil cytoplasmic antibodies) was negative. Serum C3 and serum C4 were in the normal range. Renal ultrasound was normal. She was subjected to a percutaneous ultrasound-guided renal biopsy, which revealed focal basement membrane thickening, diffuse and global mesangial proliferation, endocapillary hyper-cellularity with partial cellular crescents and mild interstitial fibrosis and tubular atrophy (<10%) [Figure 1]. Immunofluorescence revealed diffuse, global granular deposits of IgA (3+), C3c (2+), kappa and lambda chain (2+) in mesangium and glomerular capillary loops [Figure 2]. Her biopsy features were suggestive of IgA nephropathy (Oxford classification M1E1S0T0C1).
Table 1. Clinical characteristics.

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Figure 1. One of the glomeruli showing partial cellular crescent and segmental endocapillary hypercellularity. The glomerular tufts in addition show global mesangial matrix expansion and hypercellularity (H and E, ×400).

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Figure 2. There are global granular deposits predominantly in mesangium and segmentally along the glomerular capillary loops with antisera to IgA (3+).

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Case 2

A 45-year-old female was referred to us for incidental proteinuria and microscopic hematuria. She had a history of around seven episodes of unilateral painful red eye over a period of two years, considered to be recurrent anterior scleritis by her ophthalmologist. She had received topical steroids and symptomatic treatment each time. She did not have any other contributory history. Her clinical examination

.was normal except for HTN (blood pressure: 150/100 mm Hg). She was also evaluated on similar lines as that of Case 1 [Tables 1 and 2]. Her renal biopsy revealed features of IgA nephropathy (Oxford classification M0E0S1T0C1).{Table 1}
Table 2. Evaluation.
*Oxford classification for IgA nephropathy, WBC: White blood cell, ESR: Erythrocyte sedimentation rate, CRP: C-reactive protein, ACE: Angiotensin-converting enzyme, eGFR: Estimated glomerular filtration rate, ANA: Antinuclear antibodies, ANCA: Antineutrophil cytoplasmic antibodies, TSH: Thyroid stimulating hormone.


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Both the patients were given oral prednisolone (1 mg/kg/day) for six months and telmisartan (20 mg/day). On follow-up for a period of nine and six months, respectively, they remained asymptomatic and did not have further episodes of scleritis. Proteinuria remitted and renal function remained normal for both the patients.

The authors obtained all appropriate consent forms from the patients for the publication of this case report.


   Discussion Top


Patients with IgA nephropathy have wide range of clinical presentations which varies in frequency with age. In around 50% of patients with IgA nephropathy, the clinical presentation is episodic macroscopic hematuria, frequently in the second decade of life and usually following intercurrent mucosal infection. Asymptomatic urine testing identifies 30%− 40% of patients with IgA nephropathy in the most reported series.[8] Moreover, IgA nephropathy is associated with many other conditions, particularly with a number of immune and inflammatory diseases. Our case series describes its association with recurrent anterior scleritis. Scleritis is a scleral inflammatory disease which may occur as an isolated phenomenon or as a manifestation of a variety of rheumatic diseases, infections, and metabolic disorders.[9]

Way back in 1980, Nomoto et al followed up 113 patients with various primary glomerular diseases of which six patients (5%) had scleritis and all of them had IgA nephropathy.[3] Earlier, Teixeira et al published a case report of a patient with IgA nephropathy presenting with scleritis as initial presentation.[4] Our patients had recurrent episodes of scleritis over months prior to their diagnosis of IgA nephropathy although age at presentation and absence of systemic features were comparable. Garza-Leon et al have also reported a similar case.[5] A thorough evaluation for recurrent scleritis did not reveal any secondary cause per se. Identification of urinary abnormalities such as subnephrotic proteinuria with active sediments directed our further evaluation towards an underlying glomerular disorder which was subsequently diagnosed to be IgA nephropathy on renal biopsy. Urine analysis which was not a part of an initial evaluation for scleritis episodes elsewhere delayed this diagnosis, and hence the delay in further management. Abnormalities of the IgA similar to the IgA nephropathy may be involved in the development of scleritis in these patients. Sirbat et al found numerous dimeric-IgA-secreting cells in an episcleral biopsy obtained from a female patient with IgA nephropathy and frequent episodes of episcleritis, suggesting the participation of ocular mucosal immunity in some cases of IgA nephropathy.[10] We treated our patients with oral prednisolone for six months along with angiotensin receptor blocker, telmisartan. They were followed for nine and six months, respectively, after steroids were discontinued. Our line of treatment, response in terms of decrease in proteinuria and absence of further scleritis episodes was similar to the previously published case reports[3],[4] [Table 3].
Table 3. Comparison with previous studies.
ACEi: Angiotensin-converting enzyme inhibitor, ARB: Angiotensin receptor blocker, ANA: Antinuclear antibodies, ANCA: Antineutrophil cytoplasmic antibodies.


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   Conclusion Top


Recurrent scleritis can be associated with IgA nephropathy. Any patient with scleritis must be fully investigated to rule out any other systemic disease. A simple urinalysis in these patients can go a long way to detect underlying glomerulonephritis, particularly IgA nephropathy, as an early diagnosis in such cases is crucial for management. These patients respond well to steroids.

Conflicts of interest: None declared.



 
   References Top

1.
McGrogan A, Franssen CF, de Vries CS. The incidence of primary glomerulonephritis worldwide: A systematic review of the literature. Nephrol Dial Transplant 2011;26:414-30.  Back to cited text no. 1
    
2.
Pendergraft WF 3rd, Nachman PH, Jennette JC, Falk RJ. Primary glomerular disease. In: Skorecki K, Chertow GM, Marsden PA, Taal MW, Yu AS, editors. Brenner & Rector’s the Kidney. 10th ed. Philadelphia, PA, USA: Mosby Elsevier; 2016. p. 1012-89.  Back to cited text no. 2
    
3.
Nomoto Y, Sakai H, Endoh M, Tomino Y. Scleritis and IgA nephropathy. Arch Intern Med 1980;140:783-5.  Back to cited text no. 3
    
4.
Teixeira C, Quintas AM, Lopes JA, Miranda M, de Almeida E. IgA nephropathy in a patient presenting with scleritis. NDT Plus 2010;3:453-5.  Back to cited text no. 4
    
5.
Garza-Leon M, Flores D, Alarcón-Galván G, Sanchez-Martínez C. Bilateral scleritis and sclerokeratitis associated with IgA nephropathy. J Ophthal Inflamm Infect 2012;2:207-10.  Back to cited text no. 5
    
6.
Chung YM, Sheir CT, Lee SM. Scleritis in IgA nephropathy: A case report. Zhonghua Yi Xue Za Zhi (Taipei) 1995;56:279-82.  Back to cited text no. 6
    
7.
Endoh M, Kaneshige H, Tomino Y, et al. IgA nephropathy associated with myasthenia gravis and scleritis. Tokai J Exp Clin Med 1981;6:421-5.  Back to cited text no. 7
    
8.
Feehally J, Floege J. IgA nephropathy and henoch-schonlein nephritis. In: Feehally J, Floege J, Johnson RJ, editors. Comprehensive Clinical Nephrology. 3rd ed. Philadelphia, PA, USA: Mosby Elsevier; 2007. p. 253-64.  Back to cited text no. 8
    
9.
Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Surv Ophthalmol 2005;50:351-63.  Back to cited text no. 9
    
10.
Sirbat D, Saudax E, Hurault de Ligny B, Bene MC, Raspiller A. A new etiology of episcleritis: Nephropathies with IgA and/or isolated C3 deposits. J Fr Ophtalmol 1983;6:921-5.  Back to cited text no. 10
    

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Correspondence Address:
Padmanabhan Subramanian
Department of Nephrology, NU Hospitals, Bengaluru, Karnataka, India.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-2442.352442

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    Figures

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    Tables

  [Table 1], [Table 2], [Table 3]



 

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