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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2015  |  Volume : 26  |  Issue : 6  |  Page : 1262-1265
Wegner's granulomatosis developing for the first time in a patient eight years after starting maintenance hemodialysis


1 Department of Medicine, Al-Amiri Hospital, Kuwait University, Safat, Kuwait, Kuwait
2 Department of Medicine, Faculty of Medicine, Kuwait University, Safat, Kuwait

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Date of Web Publication30-Oct-2015
 

   Abstract 

Wegener's granulomatosis is a serious autoimmune disorder characterized by necrotizing small-vessel vasculitis. It is a multisystem disease that primarily affects the lung and kidneys. Previous studies indicated few relapses of vasculitis after hemodialysis due to uremic immunosuppression. Our case report describes an end-stage renal failure patient who had developed non-caseating lung granulomata with giant cell formation and fibrinoid necrosis of arterial media that is consistent with Wegner's granulomatosis for the first time and eight years after initiation of maintenance hemodialysis. We believe that such a phenomenon has rarely been reported.

How to cite this article:
Ali AR, El-Reshaid K. Wegner's granulomatosis developing for the first time in a patient eight years after starting maintenance hemodialysis. Saudi J Kidney Dis Transpl 2015;26:1262-5

How to cite this URL:
Ali AR, El-Reshaid K. Wegner's granulomatosis developing for the first time in a patient eight years after starting maintenance hemodialysis. Saudi J Kidney Dis Transpl [serial online] 2015 [cited 2020 Aug 3];26:1262-5. Available from: http://www.sjkdt.org/text.asp?2015/26/6/1262/168666

   Introduction Top


Wegener's granulomatosis is a serious autoimmune disorder characterized by necrotizing small-vessel vasculitis with autoantibodies directed against neutrophil cytoplasmic constituents, in particular proteinase 3 and myeloperoxidase. It is a multisystem disease that primarily affects the lung and kidneys. [1] Nearly 23-26% of patients will develop end-stage renal disease (ESRD) during their life-time and will require either dialysis or kidney transplantation. [2],[3] Previous studies indicated that because uremia is a chronic immunosuppressive state, patients with Wegner's granulomatosis, who already had progressed to ESRD, experienced fewer extra-renal relapses after initiating dialysis therapy. [3],[4],[5],[6] However, the development of Wegner's granulomatosis for the first time in an ESRD patient has seldom been reported.


   Case Report Top


The patient is a 44 year-old Kuwaiti female who presented with fever, cough and progressive shortness of breath over the past few months and was treated with multiple antibiotics for what was diagnosed as chest infections. She denied abdominal pain, skin rash and joint pains. The patient is on maintenance hemodialysis for the past eight years. Her primary kidney disease was progressive idiopathic focal segmental glomerulosclerosis diagnosed by kidney biopsy at the age of 14 years. It showed focal and segmental sclerosis with hyalinosis on light microscopy and deposits of IgM and C3 on the sclerotic lesions on immunoflourescent stains. At that time, she presented with anasarca, albumin at 7 g/L and massive proteinuria at 10 g/day. She had received corticosteroids and chlorambucil. She had also received lasix for edema and angiotensin converting enzyme inhibitor for hypertension and, at advanced stages, Aldomet. She was non-compliant to treatment and three years prior to initiating dialysis, she had severe secondary hyperparathyroidism. At the start of dialysis, she had a swelling of the upper jaw. A computed tomography (CT) scan of the maxilla showed a soft tissue mass with bone involvement at the left maxillary sinus. Biopsy of the mass was consistent with brown tumor of hyperparathyroidism.

On her initial physical examination, the patient was conscious and oriented. She was in distress with shortness of breath at rest. Her blood pressure was 120/80 mm Hg. She was febrile at 38°C. She did not have lymphadenopathy, goiter, jugular venous distension or edema. Systemic examination revealed coarse crepitations in the left lung, but no other significant findings. Laboratory investigations showed normal peripheral leukocyte and platelet counts. Hemoglobin was 90 g/L with normal mean corpuscular volume. Her erythrocyte sedimentation rate was 80 mm/h. Serum urea and creatinine were 23 mmol/L and 650 umol/L, respectively. Blood sugar, serum electrolytes and liver functions were normal except for albumin at 28 g/L. Serum cholesterol and thyroid-stimulating hormone were normal. Serum complements (C3 and C4) and protein electrophoresis were normal. Antinuclear antibodies, anti-ds DNA, hepatitis B surface antigen and anti-HCV antibodies were negative. Cand P-ANCA were mildly elevated. Purified protein derivative (PPD) and T-spot tests were negative. Chest X-ray showed a heterogeneous wedge-shaped lesion in the left lower lobe. The latter was confirmed using a CT scan, which showed also left pleural effusion and multiple mediastinal lymphadenopathy [Figure 1]. Bronchoscopy was performed, but failed to see specific lesions. Bronchoalveolar lavage smears were negative for acidfast bacilli and fungi. Open lung biopsy showed non-caseating granulomata with epitheliod and giant cells. There was no significant lymphocytic infiltrate [Figure 2]. Scattered arteries were seen showing segmental fibrinoid necrosis with giant cell formation. There were no lymphocytic infiltrates in those granulomata [Figure 3]. This histopathological picture was consistent with Wegner's granulomatosis Repeat paranasal scan showed normal sinuses and nasal septa with only a mild increase of the size and further ossification of the Brown's tumor, yet without any destructive element. The patient was treated with three days of 1 g of solumedrol (pulse steroid) followed by 1 mg/kg/ day of prednisone to be tapered down gradually till reaching 10 mg by the third month and 5 mg by the sixth month. In addition, she received initially 500 mg of cyclophosphamide and, after two weeks, had received another 500 mg of the drug because his peripheral leukocytic counts were normal. Subsequently, she received 1 g of cyclophosphamide IV every month for a total of six months then 1 g IV every three months for another 18 months. She improved dramatically and her lesion disappeared by one month. Subsequent treatment included prednisone 5 mg daily in addition to 1 g of IV cyclophosphamide every three months for a total period of two years.
Figure 1: Chest HRCT showing the left lower lobe triangular lesion (arrow head) prior to its biopsy.

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Figure 2: Photomicrograph of the open lung biopsy and the non-caseating granuloma (arrow head) with its epitheloid and giant cells (H&E ×100).

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Figure 3: Photomicrograph of a section of an artery in the lung lesion showing fibrinoid necrosis and inflammation with giant cell formation in its media (arrow head) that is consistent with vasculitis (H&E ×100).

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   Discussion Top


Traditionally, ESRD patients are considered immunosuppressed due to chronic uremic intoxication as evidenced by their lower antibody titers following vaccination and an inability to maintain adequate antibody titers over time. [7],[8] Moreover, the relatively low antibody response to a vaccine also appears to correlate with the degree of renal failure but not with a specific mode of dialysis. [9] It appears that disturbance in T lymphocytes and antigenpresenting cells may be responsible for the altered acquired immunity in ESRD patients [10],[11],[12] and the response in enhanced with increasing dialysis. [13] Hence, the development of lung lesions in an ESRD patient is usually linked with acute or chronic infections, malignancy and vascular complications rather than autoimmune disorders. Relapse of autoimmune diseases after establishment of ESRD is significantly less, yet has been reported. [3],[4],[5],[6] However, development of a new autoimmune disease while on dialysis, as in our patient, is rare.

Some reports indicated that kidney biopsy of some patients with Wegner's granulomatosis may show some features of focal segmental glomerulosclerosis (FSGS) yet with negative immunostains, i.e. secondary form of FSGS. [14] However, our patient had florid presentation with nephrotic syndrome and typical biopsy features of FSGS on biopsy with positive immunostains, i.e. primary FSGS. Moreover, she had negative ANCA at presentation and lacked vasculitic changes in biopsies of her maxillary bone. Finally, the long duration (30 years) of follow-up without systemic manifestations of vasculitis is also against a missdiagnosis of his original renal-limited disease as Wegner's granulomatosis and the present as a late relapse.

In conclusion, the diagnostic approach of refractory lung lesions in ESRD patients should be systematic and without the exclusion of autoimmune disease for bias of uremic immunosuppression. Open lung biopsy should be considered for definitive diagnosis.

 
   References Top

1.
Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ. Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996;7:23-32.  Back to cited text no. 1
    
2.
Weidner S, Geuss S, Hafezi-Rachti S, Wonka A, Rupprecht HD. ANCA-associated vasculitis with renal involvement: An outcome analysis. Nephrol Dial Transplant 2004;19:1403-11.  Back to cited text no. 2
    
3.
Lionaki S, Hogan SL, Jennette CE, et al. The clinical course of ANCA small-vessel vasculitis on chronic dialysis. Kidney Int 2009;76: 644-51.  Back to cited text no. 3
    
4.
Allen A, Pusey C, Gaskin G. Outcome of renal replacement therapy in antineutrophil cytoplasmic antibody-associated systemic vasculitis. J Am Soc Nephrol 1998;9:1258-63.  Back to cited text no. 4
    
5.
Elmedhem A, Adu D, Savage CO. Relapse rate and outcome of ANCA-associated small vessel vasculitis after transplantation. Nephrol Dial Transplant 2003;18:1001-4.  Back to cited text no. 5
    
6.
Gera M, Griffin MD, Specks U, Leung N, Stegall MD, Fervenza FC. Recurrence of ANCA-associated vasculitis following renal transplantation in the modern era of immunosupression. Kidney Int 2007;71:1296-301.  Back to cited text no. 6
    
7.
Rodby RA, Trenholme GM. Vaccination of the dialysis patient. Semin Dial 1991;4:102-5.  Back to cited text no. 7
    
8.
Dinits-Pensy M, Forrest GN, Cross AS, Hise MK. The use of vaccines in adult patients with renal disease. Am J Kidney Dis 2005;46:9971011.  Back to cited text no. 8
    
9.
Kausz A, Pahari D. The value of vaccination in chronic kidney disease. Semin Dial 2004;17:9-11.  Back to cited text no. 9
    
10.
Eleftheriadis T, Antoniadi G, Liakopoulos V, Kartsios C, Stefanidis I. Disturbances of acquired immunity in hemodialysis patients. Semin Dial 2007;20:440-51.  Back to cited text no. 10
    
11.
Litjens NH, Huisman M, van den Dorpel M, Betjes MG. Impaired immune responses and antigen-specific memory CD4+ T cells in hemodialysis patients. J Am Soc Nephrol 2008;19:1483-90.  Back to cited text no. 11
    
12.
Agrawal S, Gollapudi P, Elahimehr R, Pahl MV, Vaziri ND. Effects of end-stage renal disease and haemodialysis on dendritic cell subsets and basal and LPS-stimulated cytokine production. Nephrol Dial Transplant 2010;25: 737-46.  Back to cited text no. 12
    
13.
Dacko C, Holley JL. The influence of nutriational status, dialysis adequacy, and residual renal function on the response to hepatitis B vaccination in peritoneal dialysis patients. Adv Perit Dial 1996;12:315-7.  Back to cited text no. 13
    
14.
D'Agati V. Pathologic classification of focal segmental glomerulosclerosis. Semin Nephrol 2003;23:117-34.  Back to cited text no. 14
    

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Correspondence Address:
Kamel El-Reshaid
Department of Medicine, Faculty of Medicine, Kuwait University, P O Box 24923, 13110 Safat
Kuwait
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DOI: 10.4103/1319-2442.168666

PMID: 26586069

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    Abstract
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