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RENAL DATA FROM THE ARAB WORLD |
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Year : 2001 | Volume
: 12
| Issue : 4 | Page : 554-561 |
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Kidney Lesions Associated with Systemic Vasculitis: Report of 25 Cases in Morocco |
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Niang Abdou, Fatihi El Mustapha, Medkoury Ghislaine, Zahiri Khalid, Ramdani Benyounes, Benghanem Gharbi Mohamed, Hachem Khadija, S Squalli, Zaid Driss
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 2023 Feb 4];12:554-61. Available from: https://www.sjkdt.org/text.asp?2001/12/4/554/33546 |
Introduction | |  |
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 | |  |
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 | |  |
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 | |  |
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.
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Correspondence Address: Niang Abdou BP 9131 Mers Sultan, 20400 Casablanca, Morocco
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 18209403  
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