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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2001  |  Volume : 12  |  Issue : 4  |  Page : 554-561
Kidney Lesions Associated with Systemic Vasculitis: Report of 25 Cases in Morocco


Services de Néphrologie et d'Anatomie Pathologique, Ibn Rochd Hospital, Casablanca, Royaume du Morocco

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   Abstract 

In this retrospective study, we report 25 patients with renal injury caused by systemic vasculitis. These patients were hospitalized at the department of nephrology­hemodialysis in Ibn Rochd hospital from 1985 to 1998. The mean age of the patients was 36 years (range 3 to 57 years) with male predominance (68%). The clinical presentations included cutaneous purpura in 80% of the patients, arthritis in 60% and pulmonary hemorrhage in 20%. The renal injury manifested as abnormal urinary sediment in 20 of the study patients (80%), nephritic syndrome in 14 (56%), renal failure in nine (36%). Three patients (12%) had renal failure as the initial presentation followed by the other manifestations of vasculitis. The anti-neutrophil cytoplasmic antibodies (ANCA) have been checked in only six patients of whom four were positive. Henoch-Schonlein purpura was the most common clinical sign in 11 patients (44%) followed by Behcet's disease in five (20%). The treatment was variable in type and duration but generally included corticoids alone or in combination with immunosuppressants. The prognosis of the renal injury was variable. The renal function remained stable in those with Henoch-Schonlein purpura, but was less favorable with the other etiologies with more predominance of renal failure and end-stage renal disease. This retrospective analysis of our experience is given to throw light on the pattern of vasculitis in our region.

How to cite this article:
Abdou N, El Mustapha F, Ghislaine M, Khalid Z, Benyounes R, Mohamed BG, Khadija H, Squalli S, Driss Z. Kidney Lesions Associated with Systemic Vasculitis: Report of 25 Cases in Morocco. Saudi J Kidney Dis Transpl 2001;12:554-61

How to cite this URL:
Abdou N, El Mustapha F, Ghislaine M, Khalid Z, Benyounes R, Mohamed BG, Khadija H, Squalli S, Driss Z. Kidney Lesions Associated with Systemic Vasculitis: Report of 25 Cases in Morocco. Saudi J Kidney Dis Transpl [serial online] 2001 [cited 2020 Jun 2];12:554-61. Available from: http://www.sjkdt.org/text.asp?2001/12/4/554/33546

   Introduction Top


Various forms of vasculitides may affect the kidney. The classification of vasculitides was agreed upon at the 1993 Chapel Hill con­sensus conference for the nomenclature of systemic vasculitis [1] and was based on the predilection for injury of the different vascular beds. The vascular beds of one or more organs may be involved simultaneously or at different times. Some vasculitides have predilection for larges arteries (giant cell arteritis, Takayashu's arteritis), while others affect mainly medium-sized vessels (polyarteritis nodosa, Kawasaki disease) and small vessels. However, there is so much overlap in the size of the vessel involved by different vasculitides that other criteria are important for precise diagnosis, such as immune complex mediated vasculitis (Henoch-Schonlein purpura, cryoglo­bulinemic vasculitis, rheumatoid vasculitis, Behcet's disease and lupus nephritis), direct antibody mediated diseases (anti-GBM disease, Good pasture syndrome and Kawasaki disease), pauci-immune small vessel vasculitis (microscopic polyangiitis, Wegener granulomatosis, and Churg­ Strauss syndrome). [1],[2],[3]

In this report we evaluate the renal injury caused by systemic vasculitis and our experience with their outcome.


   Patients and Methods Top


This retrospective study included all patients who developed nephropathy associated with vasculitis and hospitalized at the department of Nephrology-Hemodialysis in Ibn Rochd hospital, Casablanca, Morocco, from 1985 to 1998. Vasculitis represented 3% of all the admissions to our department. There were 25 patients with mean age of 36 years (range from 3 to 57 years) and male predominance (68%). The kidney biopsies performed in these cases were examined by light microscopy. Examination of the specimens by immunofluorescence was added in 1996.


   Results Top


The clinical and pathological findings are summarized in [Table - 1]. The clinical presentations included cutaneous purpura in 80% of the patients, arthritis in 60% and pulmonary hemorrhage in 20%. The renal injury manifested as abnormal urinary sediment in 20 of the study patients (80%), nephritic syndrome in 14 (56%), renal failure in nine (36%). Three patients (12%) had renal failure as the initial presentation followed by the other manifestations of the vasculitis. The anti-neutrophil cytoplasmic antibodies (ANCA) have been checked in only six patients out of whom four were positive. Henoch-Schonlein purpura was the most common clinical sign in 11 study patients (44%) followed by Behcet's disease in five (20%).

The approach for management and prognosis were related to the disease entity as described below:

Henoch-Schonlein Purpura (HSP) (11 patients)

Oral corticosteroids (2 mg/kg/day) were the mean therapy, received by nine patients, preceded by three boluses of methyl prednisone in two cases (Case 3 and 7). A complete recovery was obtained in less than six months in one patient (Case 4), and after six months in five patients. No evolution to chronic renal failure (CRF) was observed.

Behcet's Disease(five patients)

Two patients were treated by oral cortico­steroids (1 mg/kg/d) after three boluses of methyl prednisone. Immunosuppressive drugs, cyclophosphamide followed by cyclosporin were used in Case No. 16 who died because of the severity of the disease. The other two patients remained stable on colchicine alone.

Wagener Granulomatosis (three patients)

Oral corticosteroids (1 mg/kg) after three pulses of methylprednisone (10 mg/kg/day) were administered followed by pulse of cyclophosphamide (10 mg/kg/pulse) once a month for six months followed by 3-monthly pulses for a total of 12 pulses. The course was favorable in two cases that had normal renal function and improvement of the pulmonary manifestations. Chronic renal failure was observed in Case No. 17.

Good pasture Syndrome (one patient)

Oral corticosteroids (1 mg/kg/day) after three pulses of methylprednisone and a pulse of cyclophosphamide failed to stop evolution to end stage renal disease and hemodialysis.

Cryoglobulinemia (two patients)

The two patients with this diagnosis were treated by oral corticosteroids (1 mg/kg/d) after three pulses of methylprednisone. Cyclophosphamide (one pulse per month for 6 months) was administered in one patient. After a follow-up of two years, the patient No. 20 progressed to end-stage renal disease and needed hemodialysis. The second patient with antibodies to hepatitis C virus positive did not receive antiviral therapy (interferon) due to presence of antithyro­globulin antibodies.

Polyartiritis Nodosa (one patient)

Oral corticosteroids (1 mg/kg/d) were administered after cyclophosphamide pulses (total of 12 pulses in two years). Diabetes and myocardial infarction were observed during the follow-up. The patient remained with normal renal function for three years of follow-up.

Takayashu's Disease (two patients)

The first patient (Case 24) had good control of her blood pressure after left nephrectomy and oral corticosteroids. Two years later, her disease was active again during pregnancy. The renal arteriography showed severe arterial stenosis treated by aorta-femoral prosthetic by-pass. Even­tually, the patient developed chronic renal failure. The blood pressure of the second patient (Case 25) was uncontrolled despite the right nephrectomy and multiple anti­hypertensives. Four months later, he succumbed to cerebral hemorrhage.


   Discussion Top


During the last decade, substantial improvement occurred in our understanding of the spectrum of small vessel vasculitides and specially its association with ANCA. The exact role of the antibodies in the pathogenesis of small vascular disease remains unclear. [4] The primary systemic vasculitides are uncommon diseases. Scott et al [5] estimated an incidence of 4.6 new cases/year of systemic vasculitis per million of population, based on the number of patients treated in a district hospital over a period of eight years. Current estimates are near to 15 per million per year. [6] Whether this rise is due to true increased incidence, changing referral patterns, or increased recognition of the disease is uncertain. A study in southern England suggested an annual incidence of ANCA-associated renal disease of seven per million. [7] In our study, vasculitis associated with renal diseases represented 3% of all the hospitalizations in our center over 13 years.

HSP was predominant in our study. Tissue IgA deposition is a hallmark of HSP. The nephritis that accompanies HSP may be indistinguishable histologically from IgA nephropathy. [8] In our study, the most common histological feature was focal segmental glomerulonephritis (63%). No crescents were found in our patients, which is an important prognostic factor. Half of the affected children are usually under the age of five, although renal involvement is more likely to be severe in older children. [9] Prognosis was excellent, with partial or complete recovery in all our patients, similar to the previous reports. [10] No progress to chronic renal failure was observed during our follow-up (22 months). Though there is no evidence that therapy with oral corticosteroids has any beneficial effect in patients with marked proteinuria and/or impaired renal function during the acute episode, [9],[11] two of our patients with acute renal failure received three pulses of methylprednisone followed by oral pred­nisone with favorable outcome.

Behcet's disease is predominant in the Mediterranean countries and Japan. It occurs in adults, [12] which is compatible with the mean age in our study. There are no pathognomonic laboratory tests in Behcet's disease and the diagnosis is made on the basis of clinical findings. [13],[14],[15] The most frequent kidney lesion in Behcet's disease is amyloidosis, [16] which was the major finding in our study (3/5 cases). Amyloidosis usually occurs late while focal segmental glomerulosclerosis is an early finding. [17] Significant ocular, neurological, and vascular manifestations typically require treatment with steroids and immunosuppressive such as azathioprine, cyclophosphamide, cyclos­porine or chlorambucil. We used cyclosporine after cyclophosphamide unsuccessfully in one patient with a severe form of the disease. Treatment with cyclosporine and prednisone can result in improvement in the renal and the extrarenal manifestations of the disease. [18] Non-steroidal anti-inflammatory drugs and colchicine have been used in the less aggressive cases with variable success. [17],[19] Behcet's disease has exacerbations and remissions, and may become less severe with time. [20] However, death can occur in the severe cases. [21]

The reported incidence of Wagener's granulomatosis (WG) has increased over the last decade, which may be attributed to an increased awareness of the disease among the clinicians and the introduction of assays for ANCA in 1985. [22] In the setting of a clinical picture compatible with WG, a positive c-ANCA finding is a strong circumstantial evidence for the diagnosis. [23]

The majority of our patients with WG (2/3) were c-ANCA positive. The renal involve­ment at presentation varies between 20% and 80%. Patients who have renal involve­ment and patients with generalized disease are known to have shorter life expectancies than patients with limited disease. [24] The outcome of two patients in our study was good; one kept a normal renal function (Case 18) and the other (Case 17) had mild renal insufficiency. Apart from the pulmonary hemorrhage, the presence of other pulmonary findings (pulmonary infiltrates, nodules or cavities) does not increase the risk of death. [25] Our patients did not have pulmonary hemorrhage.

There is a causal link between chronic hepatitis C viral (HCV) infection and essential mixed cryoglobulinemia (EMC). [26] One of our two patients (Case 20) with cryoglobulinemia was HCV positive, but did not receive interferon a therapy. Renal failure developed during the two years of follow-up despite the aggressive therapy (prednisone and cyclo­phosphamide). The other patient had a stable renal function after two years of follow-up (Case 21). Prognosis and response to interferon has recently been reviewed. [27]

Good pasture syndrome refers to the clinical constellation of glomerulonephritis, pulmonary hemorrhage and anti glomerular basement membrane (anti-GBM) antibodies. [28],[29] Renal biopsy is generally indicated because of rapidly progressive renal failure. Crescentic glomerulonephritis is seen on light micro­scopy and linear deposition of IgG along the glomerular capillaries and occasionally the tubules is seen on immunofluorescence microscopic examination. ANCA is positive in 10 to 38 percent of patients with anti­GBM antibody. [30] Oral corticosteroids and cyclophosphamide did not improve the prognosis of our patients who eventually needed chronic hemodialysis.

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects the small and medium-size muscular arteries. [31] Skins manifestations of PAN may include livedo reticularis, skins ulcers, vesicular eruption and purpuric lesions. [32] The latter sign was found in our patients. Glomerulonephritis appears to be more common in patients with microscopic polyarteritis than those with classic PAN. Renal arteriography often makes the diagnosis, demonstrating multiple aneurysms and irregular constrictions in the large vessels with occlusion of small penetrating arteries. [31] Most cases of PAN are idiopathic, although hepatitis B virus (HBV) infection and hairy cell leukemia are pathogenically important. [33] Our patient was HBV negative and p-ANCA positive; p-ANCA is found in 30% of cases. [34] Long-term remission can be induced in most patients with cyclo­phosphamide, even in those who have been steroid resistant. [35] Untreated generalized PAN is associated with a poor prognosis (13% five year survival). [31] The outcome has improved with modern therapy to approximately 60% at five years. [36]

Takayashu's arthritis (TA) is a chronic vasculitis with unknown etiology. The disease primarily affects the aorta and its primary branches. [37] Arteriography is usually necessary to confirm the diagnosis. The early diagnosis of TA may be difficult since early symptoms such as fatigue, malaise, joint arches and low-grade fever are non­specific. Laboratory changes reflect the inflammatory process but are mostly non­specific. [38] Angioplasty or bypass grafts may be necessary once irreversible arterial stenosis has occurred. [39] Methotrexate or cyclophosphamide are usually necessary in approximately one half of the patients with TA due to resistance to steroids. [40] Vascular involvement tends to be progressive. However, 80 to 90 percent five-year survival has been reported. [41] The prognosis in our patients was poor in spite of nephrectomy in the two cases because hypertension was not very well controlled. Thus the patient No.25 died because of hypertension complications.

 
   References Top

1.Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides: proposal of an international consensus con­ference. Arthritis Rheum 1994;37:187-92.  Back to cited text no. 1    
2.Jennette JC, Wilkman AS, Falk RJ. Anti­neutrophil cytoplasmic auto-antibody­associated glomerulonephritis and vasculitis. Am J Pathol 1989;135:921-30.  Back to cited text no. 2    
3.Falk RJ, Jennette JC. Anti-neutrophil cyto­plasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med 1988;318:1651-7.  Back to cited text no. 3    
4.Nachman PH, Falk RJ. ANCA-associated small vessel vasculitis. In: W.B. Sauders Company (ed) Brady Wilcox, therapy in nephrology and hypertension: a companion to Brenner and rector's. Kidney 1999;158-64.  Back to cited text no. 4    
5.Scott DG, Bacon PA, Elliott PJ, Tribe CR, Wallington TB. Systemic vasculitis in a district general hospital 1972-1980: clinical and laboratory features, classification and prognosis of 80 cases. QJM 1982;51:292-311.  Back to cited text no. 5    
6.Watts RA, Jollife VA, Carruthers DM, Scott DGI. Incidence of systemic vasculitis (SV) in the United Kingdom. Clin Exp Immunol 1995;101(Suppl):65.  Back to cited text no. 6    
7.Garrett PJ, Dewhurst AG, Morgan LS, Mason JC, Dathan JR. Renal disease associated with circulating anti-neutrophil cytoplasm activity. Q J Med 1992;85:731-49.  Back to cited text no. 7    
8.Glassock RJ, Cohen EH, Adler SG. Primary glomerular disease. In: Brenner BM (ed): The Kidney, 5 th ed; Philadelphia: WB Sauders 1996;1392-497.  Back to cited text no. 8    
9.Meadow SR. Henoch-Schonlein syndrome. In: Pediatric nephrology. 2nd Ed, Edelmann, CM (ed), Little, Brown, Boston 1992;1525-33.  Back to cited text no. 9    
10.Blanco R, Martinez-Taboada VM, Rodriguez­Valverde V, et al. Henoch-Schonlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheumatoid 1997;40:859.  Back to cited text no. 10    
11.Cameron JS. Henoch-Schonlein Purpura: clinical presentation. Contrib Nephrol 1984; 40:246-9.  Back to cited text no. 11    
12.Derray J. La maladie de Behcet et son epidemiologie. Sem Hop Paris 1990;66:34.  Back to cited text no. 12    
13.Meulders Q, Pirson Y, Cosyns JP, et al. Course of Henoch-Schonlein nephritis after renal transplantation. Report on ten patients and review of the literature. Transplan­tation 1994;58:1179-86.  Back to cited text no. 13    
14.Nast CC, Ward HJ, Koyle MA, Cohen AH. Recurrent Henoch-Schonlein purpura following renal transplantation. Am J Kidney Dis 1987;9:39-43.  Back to cited text no. 14    
15.Hasegawa A, Kawamura BP, Ito H, et al. Fate of renal grafts with recurrent Henoch­Schonlein purpura nephritis in children. Transplant Proc 1989;21:2130-3.  Back to cited text no. 15    
16.Auriol M, Beaufils H, Herreman G, et al. Glomerulopathy during Behcet's syndrome. Immunofluorescent and electron micro­scopy studies. Ann Med Interne Paris 1979;130:393-9.  Back to cited text no. 16    
17.Hamza M, Ben Maiz H, Ayed K, Ben Ayed H. Behcet's syndrome with amyloidosis. Report of a case. Clinical remission with colchicines. Rev Rhummal Osteoartic 1980;47:6.  Back to cited text no. 17    
18.Sajjadi H, Soheilian M, Ahmadieh H, et al. Low dose cyclosporin-A therapy in Behcet's disease. J Ocul Pharmacol 1994;10:553-60.  Back to cited text no. 18    
19.Wechler B, Le Thi Huong D, Godeau P. Le traitement de la maladie de Behcet's. Sem Hop Paris 1986;62:19.  Back to cited text no. 19    
20.O'Duffy JD. Behcet's syndrome. In: primer on the rheumatic disease, 10th Ed 1993; 29:206.  Back to cited text no. 20    
21.Park KD, Bang D, Lee ES, Lee SH, Lee S. Clinical study on death in Behcet's disease. J Corean Med Sci 1993;8:241.  Back to cited text no. 21    
22.Van der Woude FJ, Rasmussen N, Lobatto S, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet 1985;1:425-9.  Back to cited text no. 22    
23.Kerr GS, Fleisher TA, Hallahan CW, et al. Limited prognostic value of changes in antineutrophil cytoplasmic antibody titter in patients with Wegener's granulomatosis. Arthritis Rheum 1993;36:365-71.  Back to cited text no. 23    
24.Matteson EL, Gold KN, Bloch DA, Hunder GG. Long-term survival of patients with Wegener's granulomatosis from the American College of Rheumatology Wegener's Granulomatosis Classification Criteria Cohort. Am J Med 1996;101:129-34.  Back to cited text no. 24    
25.Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ. Prognostic markers in patients with anti-neutrophil cytoplasmic autoantibody-associated microscopic poly­angiitis and glomerulo-nephritis. J Am Soc Nephrol 1996;7:23-32.  Back to cited text no. 25    
26.Sansonno D, De Vita S, Iacobelli AR, et al. Clonal analysis of intrahepatic B cells from HCV-infected patients with and without mixed cryoglobulinemia. J Immunol 1998; 160:3594-601.  Back to cited text no. 26    
27.Tarantino A, Campise M, Banfi G, et al. Long-term predictors of survival in essential mixed cryoglobulinemic glomeruloneph­ritis. Kidney Int 1995;47:618-23.  Back to cited text no. 27    
28.Salant DJ. Immunopathogenesis of crescentic glomerulonephritis and lung purpura. Kidney Int 1987;32:408-25.  Back to cited text no. 28    
29.Hellmark T, Johansson C, Wieslander J. Characterization of anti-GBM antibodies involved in Goodpasture's syndrome. Kidney Int 1994;46:823-9.  Back to cited text no. 29    
30.Kalluri R, Meyers K, Mogyorosi A, et al. Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti­glomerular basement membrane disease. J Am Soc Nephrol 1997;8:1795-800.  Back to cited text no. 30    
31.Balow JE. Renal vasculitis. Kidney Int 1985;27:954-64.  Back to cited text no. 31    
32.Karlsberg PL, Lee WM, Casey DL, et al. Cutaneous vasculitis and rheumatoid factor positivity as presenting signs of hepatitis C virus-infected mixed cryoglobulinemia. Arch Dermatol 1995;131:1119.  Back to cited text no. 32    
33.Guillevin L, Lhote F, Cohen P, et al. Polyarteritis nodosa related to hepatitis B­virus. A prospective study with long-term observation of 41 patients. Medicine Baltimore 1995;74:238-53.  Back to cited text no. 33    
34.Noel LH, Lesavre P. La biopsie renale dans les vascularites et le syndrome de Good pasture. In: Droz D. La biopsie renale. Ed Inserm, Paris 1996:335.  Back to cited text no. 34    
35.Leib ES, Restivo C, Paulus HE. Immuno­suppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med 1979;67:941-7.  Back to cited text no. 35    
36.Guillevin L, Le Thi Huong DU, Godeau P, et al. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br J Rheumatol 1988;27:258-64.  Back to cited text no. 36    
37.Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayashu's arteritis: new classification. Int J Cardiol 1997;54(Suppl):155.  Back to cited text no. 37    
38.Kerr GS. Takayasu's Arteritis. Rheum Dis Clin North Am 1995;21:1041-58.  Back to cited text no. 38    
39.Ishikawa K, Maetani S. Long-term outcome for 120 Japanese patients with Takayasu's disease. Clinical and statistical analysis of related prognostic factors. Circulation 1994;90:1855-60.  Back to cited text no. 39    
40.Hoffman GS, Leavitt RY, Kerr GS, et al. Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with metho­trexate. Arthritis Rheum 194;37:578-82.  Back to cited text no. 40    
41.Eichhorn J, Sima D, Thiele B, et al. Anti­endothelial cell antibodies in Takayasu arteritis. Circulation 1996;94:2396-401.  Back to cited text no. 41    

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