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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2018  |  Volume : 29  |  Issue : 4  |  Page : 989-992
An uncommon cause of rapidly progressive renal failure in a lupus patient: Pauci-immune crescentic glomerulonephritis

1 Department of Nephrology, Jawaharlal Nehru Medical College, Sawangi, Wardha, Maharashtra, India
2 Department of Nephrology, Government Medical College and Super Specialty Hospital, Nagpur, Maharashtra, india
3 Department of Nephrology, Shravan Kidney Institute, Nagpur, Maharashtra, india
4 Department of Medicine, Northern Railway Central Hospital, New Delhi, india
5 Department of Nephrology and Clinical Transplantation, Institute of Kidney Diseases and Research Center, Dr. H. L. Trivedi Institute of Transplantation Sciences, Ahmedabad, Gujarat, India

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Date of Submission02-May-2017
Date of Acceptance03-Jun-2017
Date of Web Publication28-Aug-2018


We report a case of systemic lupus erythematosus (SLE) who presented with rapidly progressive renal failure (RPRF) with positive antinuclear antibody (ANA) and anti-double-stranded DNA (dsDNA) antibody and active urinary sediment in the form of microscopic hematuria and proteinuria. Provisional clinical diagnosis of lupus nephritis was made. Renal biopsy showed pauci-immune crescentic glomerulonephritis, the diagnosis of which was supported by positive serum anti-MPO antibody. Renal biopsy in SLE patients can sometimes reveal varied pathological entities such as antinuclear cytoplasmic antibodies (ANCAs) positive vasculitis, as in our case, which modified our treatment protocol. Thus, in a patient with SLE presenting with RPRF with active urinary sediments, ANCA serology, and renal biopsy with immunofluorescence examination should be performed always.

How to cite this article:
Balwani MR, Bawankule C, Khetan P, Ramteke V, Tolani P, Kute V. An uncommon cause of rapidly progressive renal failure in a lupus patient: Pauci-immune crescentic glomerulonephritis. Saudi J Kidney Dis Transpl 2018;29:989-92

How to cite this URL:
Balwani MR, Bawankule C, Khetan P, Ramteke V, Tolani P, Kute V. An uncommon cause of rapidly progressive renal failure in a lupus patient: Pauci-immune crescentic glomerulonephritis. Saudi J Kidney Dis Transpl [serial online] 2018 [cited 2020 Aug 7];29:989-92. Available from: http://www.sjkdt.org/text.asp?2018/29/4/989/239632

   Introduction Top

Rapidly progressive renal failure (RPRF) is characterized by a rapid loss of kidney functions over days to weeks. Immune complex-mediated glomerular disease is the usual cause of RPRF with active urinary sediment in a lupus patient, the class of which can be differentiated on renal biopsy specimen. Pauci-immune glomerulonephritis (GN) may rarely be seen in lupus patients.[1] Positive antinuclear cytoplasmic antibodies (ANCA) are seen sometimes in patients with lupus nephritis (LN) which may or may not be pathogenic.

   Case Report Top

Consent of the patient was obtained before reporting the case. A 26-year-old female patient, a known case of systemic lupus erythematosus (SLE) presented with pedal edema and gradually progressive oliguria for 15 days. She gave no history of hematuria, rash, joint pain, mouth ulcers, or seizures. On examination, she was conscious, well oriented, pale, and edematous. The blood pressure was 160/90 mm Hg with a pulse rate of 108/min and respiratory rate of 22/min. Cardiovascular and respiratory examinations were normal. Electrocardiogram showed sinus tachycardia. X-ray chest was normal. Fundoscopy showed Grade II hypertensive retinopathy. Laboratory findings were as follows: white blood cell count of 4600/ mm3, hemoglobin level of 7.1 g/dL, platelet count of 126,000/mm3, erythrocyte sedimentation rate of 42 mm/h, positive antinuclear antibody (ANA) (a titer of 120 by the enzyme immunoassay method), high anti-double-stranded DNA (ds-DNA) level (a titer of 80 by enzyme immunoassay method), complement (C3) 3 level of 102 mg/dL (n: 90–180), C4 level of 20.4 mg/dL (n: 10–40), and serum creatinine level of 3.0 mg/dL. Urinalysis revealed protein 100 mg/dL with 15–20/HPF red blood cells. Coombs test was negative. Ultrasonography revealed normal size kidneys with preserved corticomedullary differentiation and increased renal parenchymal echogenicity. Renal biopsy was performed. Provisional clinical diagnosis of LN was made, and the patient was empirically treated with three doses of 500 mg injectable methylprednisolone followed by oral omnacortil 1 mg/kg. Renal biopsy showed 26 glomeruli, of which 18 glomeruli were globally sclerosed, with 12 of these glomeruli showing circumferential fibrous crescents. In the remaining eight, five glomeruli showed circumferential fibrocellular crescents with collapsed capillary tuft [Figure 1]. The capillary lumina and Bowman's space were obliterated. However, fibrinoid necrosis was not seen. Widespread foci of tubular atrophy were noted. The interstitium showed diffuse areas of fibrosis along with diffuse mononuclear cell inflammatory infiltrate. Blood vessels were unremarkable. Immunofluorescence (IF) microscopy showed negative staining for IgG, IgA, IgM, fibrinogen, C1q, and C3. Only after this report of renal biopsy, antineutrophil cytoplasmic antibody (ANCA) test was performed and p-ANCA was found to be positive in a titer of 1:20 by using IF assay method. Enzyme-linked immunosorbent assay (ELISA) for MPO-ANCA also came positive. Following this, the patient was treated on lines of p-ANCA positive GN with steroids and mycophenolate mofetil to which she partially responded.
Figure 1: Renal biopsy showing two glomeruli with circumferential fibrocellular crescents with collapsed capillary tuft. The capillary lumina and Bowman's space are obliterated

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   Discussion Top

Renal involvement in lupus may include several kinds of pathological conditions; however, mostly, it is associated with immune complex-mediated glomerular disease. Pauci-immune LN is a very uncommon condition.[1] In pauci-immune necrotizing crescentic GN, glomerular necrosis and crescent formation occur in the absence of cellular proliferation and the presence of scant immune-complex deposition. ANCA are implicated in the patho-genesis targeting cytokine-primed leukocytes that expressed MPO or proteinase 3 (PR 3) antigens.[2]

LN is an immune complex-mediated renal disease, where the formation of glomerular immune deposits results in complement activation, leukocyte infiltration, cytokine release, cellular proliferation, crescent formation, and necrosis.[3] There are few reported cases of LN, in which, focal or diffuse glomerular necrosis and crescents occur without substantial subendothelial deposits.[4] Approximately 20% of patients with SLE have ANCA positivity by IF microscopy, mainly with a perinuclear (pANCA) pattern. Antinuclear antibody sero-positivity by ELISA is less frequent, and target antigens are commonly lactoferrin, cathepsin G, and MPO.[5] In our case, ANCA came positive by using both methods, IF and ELISA.

Galeazzi et al evaluated 566 patients with SLE and found ANCA positivity by IF microscopy in 16.4% (15.4% p-ANCA and 1% cANCA pattern). However, using ELISA, 9.3% had MPO-ANCA positivity and 1.7% had PR3-ANCA positivity.[6]

There is difficulty in distinguishing p-ANCA from ANA by IF microscopy.[7] Antinuclear antibody positivity has been associated with the presence of nephritis, particularly diffuse proliferative LN, as well as anti-dsDNA antibodies.[8]

While reports have failed to prove a correlation between ANCA and organ involvement,[9] Nasr et al evaluated a cohort of 10 patients with SLE, ANCA positivity, and renal biopsy findings of LN and ANCA-associated GN. All biopsies exhibited necrosis and crescents with no or rare subendothelial deposits.[7] Nine patients had p-ANCA positivity by using IF. The high incidence of MPO-ANCA sero-positivity in patients with SLE raises the possibility that one condition might trigger the other one or vice versa.

It has also been suggested that SLE may facilitate MPO autoantibody formation by promoting neutrophil degranulation and priming neutrophils to increase surface expression of MPO.[7]

The correlation between the presence of ANCA in SLE and clinical features is not clear. Some reports show no correlation between organ involvement and the presence of ANCA, while others report a link.

Here, we report a patient who presented with RPRF with positive lupus serology and active urinary sediment. Initially, due to cost restraints, complement levels, and ANCA profile were not performed, and provisional diagnosis of LN was made and treated on the same lines. Only after renal biopsy, a diagnosis of pauci-immune crescentic GN was made and subsequently, the patient was found to be p-ANCA positive with normal serum complement levels. In our case, it is probable that lupus serology represented an autoimmune response to the antinuclear antibody activity. Furthermore, the fact that, she had normal serum complement levels goes against activation of immune complexes due to SLE. On the other hand, the possibility of simultaneous ANCA/LN involvement represents an interesting hypothesis.

   Conclusion Top

In a patient, who presents with RPRF with positive lupus serology and normal serum complement levels with active urinary sediment, one should have high suspicion of ANCA-mediated renal involvement. Proper vigilance and timely diagnosis of ANCA-mediated vasculitis in a lupus patient will lead to early initiation of proper disease-oriented treatment.

Conflicts of interest: None declared.

   References Top

Akhtar M, al-Dalaan A, el-Ramahi KM. Pauci-immune necrotizing lupus nephritis: Report of two cases. Am J Kidney Dis 1994;23:320-5.  Back to cited text no. 1
Jennette JC, Xiao H, Falk RJ. Pathogenesis of vascular inflammation by anti-neutrophil cytoplasmic antibodies. J Am Soc Nephrol 2006;17:1235-42.  Back to cited text no. 2
Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 2004;65:521-30.  Back to cited text no. 3
Schwartz MM, Roberts JL, Lewis EJ. Necro-tizing glomerulitis of systemic lupus erythematosus. Hum Pathol 1983;14:158-67.  Back to cited text no. 4
Sen D, Isenberg DA. Antineutrophil cyto-plasmic autoantibodies in systemic lupus erythematosus. Lupus 2003;12:651-8.  Back to cited text no. 5
Galeazzi M, Morozzi G, Sebastiani GD, Bellisai F, Marcolongo R, Cervera R, et al. Anti-neutrophil cytoplasmic antibodies in 566 European patients with systemic lupus erythe-matosus: Prevalence, clinical associations and correlation with other autoantibodies. European concerted action on the immunogenetics of SLE. Clin Exp Rheumatol 1998;16:541-6.  Back to cited text no. 6
Nasr SH, D'Agati VD, Park HR, Sterman PL, Goyzueta JD, Dressler RM, et al. Necrotizing and crescentic lupus nephritis with antineutrophil cytoplasmic antibody seropositivity. Clin J Am Soc Nephrol 2008;3:682-90.  Back to cited text no. 7
Chin HJ, Ahn C, Lim CS, Chung HK, Lee JG, Song YW, et al. Clinical implications of antineutrophil cytoplasmic antibody test in lupus nephritis. Am J Nephrol 2000;20:57-63.  Back to cited text no. 8
Nishiya K, Chikazawa H, Nishimura S, Hisakawa N, Hashimoto K. Anti-neutrophil cytoplasmic antibody in patients with systemic lupus erythematosus is unrelated to clinical features. ' Clin Rheumatol 1997;16:70-5.  Back to cited text no. 9

Correspondence Address:
Dr. Manish R Balwani
Department of Nephrology, Jawaharlal Nehru Medical College, Sawangi, Wardha - 442,001, Maharashtra
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DOI: 10.4103/1319-2442.239632

PMID: 30152441

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