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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 3  |  Issue : 3  |  Page : 241-244

Non-lupus glomerulonephritis in a patient with systemic lupus erythematosus


1 Department of Nephrology, Theodor Bilharz Research Institute, Cairo, Egypt
2 Department of Internal Medicine, Nephrology Unit, Al-Adan Hospital, Kuwait
3 Department of Internal Medicine, Al-Adan Hospital, Kuwait
4 Department of Internal Medicine, Rheumatology Unit, Al-Adan Hospital, Kuwait

Date of Web Publication3-Aug-2015

Correspondence Address:
Emad Abdallah
Department of Nephrology, Theodor Bilharz Research Institute, Cairo
Egypt
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DOI: 10.4103/1658-631X.162040

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  Abstract 

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect almost any organ system. Lupus nephritis (LN) is one of the most serious manifestations of SLE. The occurrence of nonlupus glomerulopathies has been rarely reported in patients with SLE. Here, we report a case of male Kuwaiti patient suffering from SLE with microscopic hematuria and nonnephrotic-range proteinuia. The renal biopsy findings showed IgA nephropathy. Although the incidence of IgA nephropathy is high in Kuwait, we believe that this is the first report of a Kuwaiti patient in whom SLE presented with IgA nephropathy rather than LN.

  Abstract in Arabic 



ملخص البحث :
مرض الذئبة الحمراء هو احد أمراض المناعة الذاتية المزمنة الذي قد يؤثر على معظم أعضاء الجسم. ويعتبر التهاب الكلية المناعي من أهم المضاعفات لدى مرضى الذئبة الحمراء, كما يندر حدوث التهاب الكلى المناعي غير المصاحب لمرضى الذئبة الحمراء. يناقش الباحثون حالة مريض يعاني من مرض الذئبة الحمراء مع وجود دم وزلال في البول. بينت نتائج الخزعة من الكلية اعتلالا يدعى IgA nephropathy وليس بسبب الذئبة الحمراء.

Keywords: IgA nephropahy, lupus nephritis, systemic lupus erythematosus


How to cite this article:
Abdallah E, Al-Helal B, Asad R, Shehab F, Al-Rashidi A. Non-lupus glomerulonephritis in a patient with systemic lupus erythematosus. Saudi J Med Med Sci 2015;3:241-4

How to cite this URL:
Abdallah E, Al-Helal B, Asad R, Shehab F, Al-Rashidi A. Non-lupus glomerulonephritis in a patient with systemic lupus erythematosus. Saudi J Med Med Sci [serial online] 2015 [cited 2022 Jan 26];3:241-4. Available from: https://www.sjmms.net/text.asp?2015/3/3/241/162040


  Introduction Top


Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect almost any organ system; thus, its presentation and course are highly variable, ranging from indolent to fulminant.

The diagnosis of SLE is based on a combination of clinical findings and laboratory evidence. The presence of 4 of the 11 American College of Rheumatology (ACR) criteria yields a sensitivity of 85% and a specificity of 95% for SLE. [1],[2]

When the Systemic Lupus International Collaborating Clinics group revised and validated the ACR SLE classification criteria in 2012, they classified a person as having SLE in the presence of biopsy-proven lupus nephritis (LN) with antinuclear antibody (ANA) or anti-dsDNA antibodies or if four of the diagnostic criteria, including at least one clinical and one immunologic criterion, have been satisfied. [3]

Lupus nephritis is one of the most serious manifestations of SLE and histologically evident in most patients with SLE, even those without clinical manifestations of renal disease. [4]


  Case report Top


A 43-year-old male Kuwaiti patient presented to our renal clinic because of persistent microscopic hematuria and proteinuria. There was arthralgia, but no skin rash. His past history was unremarkable with no history of recent upper respiratory infection or gastroenteritis, and physical examination was normal. White blood count was 6.56 × 10 7 /L, hemoglobin 132.00 mmol/L, hematocrit 0.37 L/L, platelet 340.00 × 10 7 /L. Erythrocyte sedimentation rate was 60 mm/h. Chemistry values showed serum creatinine 119 mmol/L, blood urea nitrogen 31.1 mmol/L, serum potassium 5.5 mmol/L, serum sodium 131 mmol/L and albumin 32 g/L. Total Ca 2.25 mmol/L, phosphorous 1.39 mmol/L. Total cholesterol 7.4 mmol/L, high density lipoprotein-cholesterol 2.05 mmol/L and triglycerides 2.38 mmol/L. Liver enzymes were normal. The serological examination revealed circulating IgG 12.80 g/L (normal range: 7-16 g/L); IgM 0.94 g/L (normal range: 0.56-3.52 g/L); IgA 5.21 g/L (normal range: 0.7-3.12 g/L). Serum complement levels were within normal range. ANA was positive (1/640 IU/mL), anti-dsDNA antibodies were positive (48.2 IU/mL). IgG and IgM anticardiolipin antibodies were negative. Hepatitis B surface antigen and hepatitis C antibodies were negative. The urine sediment contained 40-50 red blood cells and 2-3 white blood cell/hpf and 24 urinary protein was 2405 mg/day [Table 1]. Abdominal ultrasound revealed normal both kidneys in position, size and echotecture with normal corticomedullary differentiation. Chest X-ray, electrocardiography and echocardiography were normal. The clinical manifestations in combination with ANA, anti-dsDNA antibodies were diagnostic for SLE. Renal biopsy was performed in order to establish the diagnosis and adequate therapy.
Table 1: Time course of laboratory characteristics of our patient

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Kidney biopsy findings

Light microscopy sections showed 20 glomeruli, 25% out of which showed increased cellularity [Figure 1]. Two glomeruli showed global sclerosis and one with segmental sclerosis are also seen. Interstitial inflammation and fibrosis (30%) with areas of tubular atrophy (20%) were also seen. By immunohistochemistry, the glomeruli revealed granular deposits of IgA in the glomerular basement membrane and mesangium [Figure 2]. The glomeruli were negative for IgG, IgM, C3 and C1q. The diagnosis was IgA nephropathy; Class 111 (focal proliferative glomerulonephitis).
Figure 1: Light microscopy of a glomerulus from a patient with IgA nephropathy showing increased mesangial matrix and cellularity.

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Figure 2: Immunofluorescence microscopy demonstrating large mesangial IgA deposits diagnostic of IgA nephropathy.

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Our patient was treated with prednisolone 30 mg daily for 2 months and then tapered to 20 mg daily, lisinopril 20 mg daily, Omega 3 plus twice a day and atorvastatin 20 mg daily and with follow-up, the proteinuria decreased from 2405 mg/day to 420 mg/day and still on close follow-up [Table 1].


  Discussion Top


IgA nephropathy is the most common cause of glomerulonephritis (GN) in the world. [5],[6],[7] Clinical features range from asymptomatic hematuria to rapidly progressive GN. Pathologically, a spectrum of glomerular lesions can be seen, but mesangial proliferation with prominent IgA deposition is observed in almost all biopsies.

Although IgA nephropathy is a limited nonsystemic renal disease, many systemic diseases are sporadically associated with mesangial IgA deposition. Henoch-Schönlein purpura, systemic illness, has been closely linked to IgA nephropathy. Other systemic diseases in which mesangial deposits of IgA are regularly observed include SLE, hepatitis, dermatitis herpetiformis, and ankylosing spondylitis.

The occurrence of nonlupus glomerulopathies has been rarely reported in patients with SLE. Here, we report a case of male Kuwaii patient suffering from SLE with microscopic hematuria and nonnephrotic-range proteinuria. The renal biopsy findings showed IgA nephropathy.

The pathological distinction between IgA nephropathy and LN should be straightforward because it determines the mode of treatment and the renal prognosis.

There are six classes of LN according to the classification of LN revised by the International Society of Nephrology and the Renal Pathology Society in 2003 and is based on light microscopy, immunofluorescence, and electron microscopy findings from renal biopsy specimens. [8]

These typical LN classes are characterized by staining positively for IgG, IgM, C3, C4 and C1q in the glomerular capillary basement membrane under immunofluorescent microscopy. [9]

In IgA nephropathy, pathologically, a spectrum of glomerular lesions can be seen, but mesangial hypercellularity with prominent IgA deposition is observed in almost all biopsies. [6],[7]

In our case, immunohistochemistry of the kidney biopsy, the glomeruli showed granular deposits of IgA in the glomerular basement membrane and mesangium. The glomeruli are negative for IgG, IgM, C3 and C1q.These features of IgA nephopathy (IgAN) do not match with the typical LN.

Therefore, pathologiclly this nonlupus form of glomerulonephrtis should be considered as IgA nephropathy in the patient with SLE rather than LN, because the prominent IgA deposits in the mesagium dominated the histological picture, and significant proliferation was absent. However, according to the recommendations of Churg et al. [10] Class IV LN is defined by diffuse distribution of subendothelial deposits regardless of the pattern of proliferation and the presence of large subendothelial depositions of immunoglobulins without activation of the complement system and proliferation is exceptional and although C1q, C3, C4, and IgG depositions are more common in typical LN, IgA deposition can also be seen. [9] Thus, our case with predominant mesangial IgA deposits may be proposed to be a special subtype of LN.

Serum IgA levels are elevated in approximately half of patients with IgA nephropathy, but that increase is unlikely to play a role in the pathogenesis of the disease, as markedly elevated IgA levels are observed in patients with AIDS who do not have IgA nephropathy. However, IgA is probably accumulated and deposited because of a systemic abnormality rather than a defect intrinsic to the kidney. [11]

In our case, IgA was measured in the serum of our patient and was abnormally high. Serum complement C3 and C4 levels were within normal range. As stated above, the high serum IgA level and normal complement C3, C4 support the diagnosis of IgA nephropathy.

To our knowledge, there is a few description in the literature of IgA nephropathy in patients with a well-established SLE in whom the renal biopsy findings are diagnostic for IgA nephropathy have been reported. [12],[13],[14],[15],[16],[17] In all these cases, the renal biopsy showed a mild mesangial hypercellularity with almost exclusively deposits of IgA. In these cases, the relationship between SLE and IgAN was discussed, and the result is still controversial. Most authors concluded that the reported IgAN as a complication of SLE when the two diseases coexist. [12],[13],[16]

Some other authors assumed that IgAN may be a special clinical subtype of SLE. [15],[18] Horino et al. [15] have reported a case of a male SLE patient whose renal biopsy was established as Class II LN. He was given the second renal biopsy because of repeated proteinuria, and the result suggested IgAN. The authors proposed that IgAN may be a special clinical subtype of SLE. As there is a mutual transition among the types of typical LN, typical LN may also convert into IgAN.

Furthermore, Hongyan et al. [18] reported 5 cases with SLE in whom, renal biopsy showed that all cases had mainly IgA deposits and were free of IgG, C1q, and fibrinogen-related antigen deposits under immunofluorescent microscopy, which did not match with typical LN and concluded that nephritis with mainly IgAN deposits, as an atypical LN, may be a special subtype of SLE.In conclusion, this patient may represent a rare case of IgAN in patient with SLE. Our case highlights the importance of renal biopsy in SLE patients with urinary alterations since a correct diagnosis would permit the most appropriate treatment to be started, thus avoiding unnecessary immunosuppressive treatments. The proposal that this case with predominant mesangial IgA deposits may be a special subtype of LN needs more clinical observation and research.



 
  References Top

1.
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997;40:1725.  Back to cited text no. 1
    
2.
American College of Rheumatology. 1997 Update of the 1982 American College of Rheumatology revised criteria for classification of systemic lupus erythematosus. Available from: http://www tinyurl.com/1997SLEcriteria. [Last accessed on 2013 Nov 01].  Back to cited text no. 2
    
3.
Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677-86.  Back to cited text no. 3
    
4.
Condon MB, Ashby D, Pepper RJ, Cook HT, Levy JB, Griffith M, et al. Prospective observational single-centre cohort study to evaluate the effectiveness of treating lupus nephritis with rituximab and mycophenolate mofetil but no oral steroids. Ann Rheum Dis 2013;72:1280-6.  Back to cited text no. 4
    
5.
Glassock RJ. IgA nephropathy: Challenges and opportunities. Cleve Clin J Med 2008;75:569-76.  Back to cited text no. 5
    
6.
Working Group of the International IgA Nephropathy Network and the Renal Pathology Society, Cattran DC, Coppo R, Cook HT, Feehally J, Roberts IS, et al. The Oxford classification of IgA nephropathy: Rationale, clinicopathological correlations, and classification. Kidney Int 2009;76:534-45.  Back to cited text no. 6
    
7.
Working Group of the International IgA Nephropathy Network and the Renal Pathology Society, Coppo R, Troyanov S, Camilla R, Hogg RJ, Cattran DC, et al. The Oxford IgA nephropathy clinicopathological classification is valid for children as well as adults. Kidney Int 2010;77:921-7.  Back to cited text no. 7
    
8.
Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol 2004;15:241-50.  Back to cited text no. 8
    
9.
Verma RN, Banerjee AK, Bhattacharya K. Lupus nephritis - light, electron and immunofluorescent microscopy (a clinicopathological study). Indian J Pathol Microbiol 1989;32:246-51.  Back to cited text no. 9
    
10.
Churg J, Bernstein J, Glassock RJ. Renal Diseases, Classification and Atlas of Glomerular Diseases. New York: Igaku-Shoin; 1995.  Back to cited text no. 10
    
11.
Hahn WH, Suh JS, Cho BS, Kim SD. The enabled homolog gene polymorphisms are associated with susceptibility and progression of childhood IgA nephropathy. Exp Mol Med 2009;41:793-801.  Back to cited text no. 11
    
12.
Basile C, Semeraro A, Montanaro A, Giordano R, De Padova F, Marangi AL, et al. IgA nephropathy in a patient with systemic lupus erythematosus. Nephrol Dial Transplant 1998;13:1891-2.  Back to cited text no. 12
[PUBMED]    
13.
Fujikura E, Kimura T, Otaka A, Arima S, Satoh H, Itoh S, et al. IgA nephropathy in the patient with systemic lupus erythematosus in remission. Nihon Naika Gakkai Zasshi 2002;91:3282-4.  Back to cited text no. 13
    
14.
Corrado A, Quarta L, Di Palma AM, Gesualdo L, Cantatore FP. IgA nephropathy in systemic lupus erythematosus. Clin Exp Rheumatol 2007;25:467-9.  Back to cited text no. 14
    
15.
Horino T, Takao T, Terada Y. IgA nephropathy in a patient with systemic lupus erythematosus. Lupus 2010;19:650-4.  Back to cited text no. 15
    
16.
Kobak S, Hudaverdi O, Keser G, Oksel F. Coexistence of systemic lupus erythematosus, Hashimoto's thyroiditis and IgA nephropathy in the same patient. Mod Rheumatol 2011;21:89-91.  Back to cited text no. 16
    
17.
Mac-Moune Lai F, Li EK, Tang NL, Li PK, Lui SF, Lai KN. IgA nephropathy: A rare lesion in systemic lupus erythematosus. Mod Pathol 1995;8:5-10.  Back to cited text no. 17
    
18.
Hongyan L, Yi Z, Bao D, Yuewu L, Juan M. A study on clinical and pathologic features in lupus nephritis with mainly IgA deposits and a literature review. Clin Dev Immunol 2013;2013:289316.  Back to cited text no. 18
    


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