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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2011  |  Volume : 22  |  Issue : 2  |  Page : 349-351
An unusual association of anti-GBM diseases and lupus nephritis presenting as pulmonary renal syndrome

1 Department of Nephrology, SVIMS, Tirupati, Andhra Pradesh, India
2 Department of Pathology, SVIMS, Tirupati, Andhra Pradesh, India

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Date of Web Publication18-Mar-2011

How to cite this article:
Yadla M, Krishnakishore C, Reddy S, Naveen P S, Sainaresh V V, Reddy M K, Sivakumar V. An unusual association of anti-GBM diseases and lupus nephritis presenting as pulmonary renal syndrome. Saudi J Kidney Dis Transpl 2011;22:349-51

How to cite this URL:
Yadla M, Krishnakishore C, Reddy S, Naveen P S, Sainaresh V V, Reddy M K, Sivakumar V. An unusual association of anti-GBM diseases and lupus nephritis presenting as pulmonary renal syndrome. Saudi J Kidney Dis Transpl [serial online] 2011 [cited 2020 Jun 6];22:349-51. Available from: http://www.sjkdt.org/text.asp?2011/22/2/349/77633
To the Editor,

A 32-year-old male, a known hypertensive of 2 years duration, a chronic smoker of 3-4 pack years, presented with hematuria, oliguria and worsening dyspnea of 4 days duration. He pre­sented to a local hospital with pulmonary ede­ma requiring mechanical ventilation and was then referred to our hospital for further ma­nagement. At admission, his BP was 200/110 mmHg and fundus showed features of hyper­tensive retinopathy. Chest examination revealed bilateral crepitations. On evaluation, his hemo­globin was 10.3 g/dL, total leukocyte count was 11,800/mm 3 , platelets were 168,000/mm 3 , and erythrocyte sedimentation rate (ESR) was 93 mm at the end of 1st hour. Urine analysis revealed 3+ albuminuria and plenty of RBCs. His blood urea was 168 mg/dL and serum creatinine was 10.8 mg/dL. Chest X-ray was suggestive of pulmonary edema. Echocardio­gram revealed left ventricular hypertrophy along with grade 1 diastolic dysfunction. USG abdomen showed normal sized kidneys with grade 2 echogenicity. In view of severe renal failure, he was initiated on hemodialysis. After six sessions of hemodialysis, renal biopsy was done. Renal histopathology showed 10 glome­ruli of which 1 was sclerosed. The remaining glomeruli showed endothelial proliferation, membranous thickening and a solitary glome­rulus with mild cellular crescent. There was sig­nificant interstitial infiltrate and the arterioles showed hyaline arteriosclerosis [Figure 1], [Figure 2], [Figure 3].
Figure 1: Light microscopy (H&E): Endothelial proliferation, membranous thickening.

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Figure 2: Light microscopy (H&E): Hyaline arteriosclerosis is present.

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Figure 3: Light microscopy (H&E). Sclerotic glomerulus along with significant interstitial infiltrate.

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Immunofluorescence (IF) showed linear IF along the ca-pillary wall for IgG3+, IgA2+, IgM1+, granu-lar pattern of C3+, along with C1q positivity, thus suggesting linear IF with full house pat-tern. Serology evaluation for antinuclear anti-body (ANA) was found to be negative, and anti-dsDNA was found to be positive. Anti-glomerular basement membrane (GBM) titer was also found to be high (30 IU/mL) (normal reference <12 IU/mL).

In view of the presence of anti-GBM anti­body positivity and the linear IF pattern in re­nal biopsy, along with pulmonary involvement, Goodpasture's syndrome was considered. In addition, positive anti-dsDNA and renal biopsy showing full house IF picture along with posi­tive C1q led to the diagnosis of lupus nephritis class IV in addition to Goodpasture's disease. Though it is conjectural whether anti-GBM preceded lupus or lupus preceded anti-GBM or both occurred simultaneously, the renal histo­pathologic changes suggested preexisting renal disease, presumably lupus nephritis.

The patient's clinical condition improved with­out the need for plasmapheresis and responded to the regimen of pulse methylprednisole fol­lowed by oral steroids and pulse cyclophos­phamide. During follow-up, his serum creatI­nine stabilized at 4.2 mg/dL.

Lupus nephritis with anti-GBM disease is a rare entity. Richards et al reported a male lu­pus patient with circulating anti-GBM antibo­dies and linear IF. [1] Circulating anti-GBM an­tibodies were detected in a cohort of Chinese lupus nephritis patients in the study by Li et al. [2]

Review of literature revealed different sup­positions of pathogenesis of this association, which are as follows:

Glomerular immune deposits result from im­mune interaction either between antibodies and native components of glomeruli or bet­ween antibodies and nonglomerular antigens from circulation. [3]

  1. It was assumed that in pulmonary renal syndrome with pulmonary hemorrhage, the basement membrane of the lung or kidney was damaged first by interstitial pneumonitis due to systemic lupus erythematosus (SLE) or lu­pus nephritis, and the basement membrane antigens were exposed, with secondary pro­duction of anti-GBM antibody. [4]
  2. Although experimental anti-GBM nephritis and spontaneous lupus nephritis differ in the nature of the inciting antibodies and the loca­lization of the immune deposits, pathology in both the settings may be mediated by a shared net-work of downstream molecular pathways, including complement- and FcR-dependent ac­tivation of resident renal cells and infiltrating leukocytes. T cells mediate the proinflamma­tory pathway initially and profibrotic molecules mediate later in disease. [2]
  3. In a study by Kui Liu et al, [5] kallikrein was identified as an additional molecule that ap­pears to impact both diseases concordantly, particularly KLK1 and the KLK3 promoter, when DNA single-nucleotide polymorphisms (SNPs) from independent cohorts of SLE patients and controls were compared. This study suggested that kallikreins are protective disease-associated genes in anti-GBM antibody- induced nephritis and lupus.
In conclusion, our patient had two diseases coexisting and it is hard to suggest that either one of them (lupus nephritis or anti-GBM di­sease) was the first to occur. Prevailing histo­logic changes and serology, however, were spe­culative for lupus nephritis as the preexisting disease. We report this case in view of its ra­rity and the therapy outcome in making a dia­lysis-dependent individual dialysis independent.

   References Top

1.Richards NT, Lueck C, Davies DR, Jones NF, Alkhader A, Coode P. Antiglomerular basement membrane antibody and linear immunofluo­rescence in a patient with systemic lupus ery­thematosus Clin Nephrol 1998;30:115-6.  Back to cited text no. 1
2.Li CH, Li YC, Xu PS, Hu X, Wang CY, Zou GL. Clinical significance of Antiglomerular base­ment membrane antibodies in a cohort of Chinese patients with lupus nephritis. Scand J Rheumatol 2006;35:201-8.  Back to cited text no. 2
3.Berden JH. Lupus nephritis. Kidney Int 1997; 52:538-58.  Back to cited text no. 3
4.Yamazaki K, Fujita J, Doi I, Abe S, Kawakami Y, Fukazawa Y. A case of acute pulmonary he­morrhage and positive anti-glomerular basement membrane antibody in systemic lupus erythematosus [Article in Japanese]. Nihon Kyobu Shikkan Gakkai Zasshi 1993;31(2): 251-6.  Back to cited text no. 4
5.Liu K, Li QZ, Delgado-Vega AM, et al. Kallikrein genes are associated with lupus and glomerular basement membrane-specific anti­body-induced nephritis in mice and humans. J Clin Invest 2009;119(4):911-23.  Back to cited text no. 5

Correspondence Address:
Manjusha Yadla
Department of Nephrology, SVIMS, Tirupati, Andhra Pradesh
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PMID: 21422643

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