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Summary : Renal disease is common in systemic lupus erythematosus (SLE) and significantly influences patient prognosis. Immunosuppressive therapy has markedly improved outcome, however, it increases the risk of infection and cancer induction. Although several therapeutic regimens have proved to be effective in controlling lupus nephritis (LN), optimal therapy is still a matter of discussion. The following review summarizes our current knowledge in treating LN and discusses new aspects in pathogenesis. Hopefully, continuing progress in uncovering details about the pathogenesis of SLE might lead to more disease­specific approaches to treat the underlying immunological disorder.

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Minimal Change Disease (MCD) is the lesion most commonly associated with nephrotic syndrome in children, accounting for over 75% of cases. Although less common, MCD still accounts for up to 30% of adult onset nephrotic syndrome. Unlike children, in whom MCD is primarily idiopathic, secondary causes of MCD are seen in 13% of adults and must be considered, as the therapeutic approach to these patients is defined by the underlying cause. Clinical features at presentation in nephrotic adults with MCD can include microscopic hematuria, hypertension, and renal insufficiency, making MCD indistinguishable clinically from focal segmental glomerulosclerosis. As a result, a renal biopsy is required in adults in order to correctly diagnose and manage the nephrotic syndrome. As in children, response to therapy leads to a complete remission of proteinuria in up to 97% of adults, although, adults require a more prolonged course of therapy (16-28 weeks) compared to children (8 weeks). Relapse of MCD is extremely common in children (71%) and can be seen in up to 85% of adult patients. Relapses occur more frequently in younger adults (<45 years) and are often seen in the first 6-12 months after the onset of a remission. Successful treatment of relapses can often be achieved with a second course of steroids. However, up to 50% of relapsing adults become frequent relapsers or steroid dependent. In these patients, a stable remission can be induced by treatment with either cyclophosphamide or cyclosporine. Overall, the long-term outcome of adult onset MCD is excellent, with fewer than 5% of patients progressing to end-stage renal disease and a patient survival of 83%-98% at 15 years.

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Summary: Treatment options for IgA nephropathy (IgAN) must take into consideration the pathophysiology, namely the role of eicosanoids, angiotensin II and monocyte­macrophages releasing reactive oxygen species (ROS). Angiotensin converting enzyme inhibitors and early corticosteroid usage are prime therapies. Tonsillectomy should be considered. Other components of a therapeutic cocktail could be; (a) thromboxane antogonist, (b) leukotriene antagonist, (c) platelet activating factor antagonist, (d) an anti­oxidant and (e) an anti-fibrotic agent like pentoxyfylline. In the new millenium, there will be focus on anti-proliferative measures like platelets dependent growth factor aptamers. For unusual cases with rapid progression, the use of FK-506 or mycophenolate mofetil can be considered.

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Hepatitis C virus (HCV) may have a pathogenic role in several forms of immune complex glomerulonephritis (ICGN), including cryoglobulinemic membrano­proliferative glomerulonephritis (MPGN) and membranous nephropathy. HCV infection may also be related indirectly (e.g. secondary to HCV-related liver disease) or coincidentally to glomerulonephritis. These include cases of fibrillary/immunotactoid glomerulopathy, MPGN arising in allografts, allograft glomerulopathy, rapidly progressive glomerulo­nephritis, focal and segmental glomerulosclerosis, and ICGN arising in individuals co­infected with human immunodeficiency virus (HIV). This review summarizes the clinical and pathologic features of HCV-associated glomerular disease, particularly immune complex glomerulonephritis, and discusses possible pathogenic mechanisms.

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Although not all renal disease that diabetic patients develop is due to diabetic glomerulosclerosis, the great majority of patients progressing to advanced renal failure suffer from diffuse or nodular (Kimmelstiel Wilson's) diabetic glomerulosclerosis. This condition has become the single most frequent cause of end-stage renal failure in the Western world. Recent studies indicate that an interplay between genetic predisposition and other factors such as hyperglycemia, blood pressure, age, gender, smoking and ethnicity, predispose to nephropathy both in type 1 and type 2 diabetes mellitus. It has also become clear that trace albuminuria ("microalbuminuria") provides a unique opportunity to recognize incipient renal involvement early on, particularly in type 1 and less specifically in type 2 diabetes. Increasing evidence indicates that early intervention delays progression of nephropathy. Factors which promote progression of nephropathy include hypertension, proteinuria, smoking, poor glycemic control and, less certainly, high dietary protein intake and hyperlipidemia. The most important strategies to combat the medical catastrophe of increasing numbers of diabetic patients with end-stage renal failure include (i) prevention of diabetes (mainly type 2), (ii) glycemic control to prevent onset of renal involvement and (iii) meticulous antihypertensive treatment to avoid progression of nephropathy. Survival of diabetic patients on dialysis and after transplantation is inferior to that of non-diabetic patients, mainly because of high rate of cardiovascular death. There is consensus that in the absence of major vascular disease the best treatment is renal transplantation in the type 2 diabetic patient and combined kidney and pancreas transplantation in the type 1 diabetic patient.

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To obtain a more recent and comprehensive insight into the prevalence of glomerular diseases in our patient population, medical records of 200 patients with biopsy proven glomerulonephritis (GN), between January 1994 and June 1999, at the King Khalid University Hospital, Riyadh, Saudi Arabia were analyzed. Primary glomerular disease was found to be the most prevalent, accounting for 63.5% of all glomerular diseases. Among primary glomerular diseases, focal and segmental glomerulosclerosis (FSGS) was the most common histological lesion (34.6%) and was associated with a high prevalence of hypertension (86.4%), nephrotic syndrome (68.18%), hematuria (63.6%) and renal functional impairment (27.3%). Mesangioproliferative GN was the second most common lesion (25.1%) followed by mesangiocapillary GN (15.7%), IgA nephropathy (10.2%), and minimal change disease (8.5%). Amongst secondary glomerular diseases, lupus nephritis was the most prevalent (24.5%). In conclusion, primary glomerular diseases constituted the commonest group encountered and the prevalence of FSGS was quite high with male sex and young adults predominating. FSGS was also associated with a high prevalence of end­stage renal disease. Further collaborative studies are necessary to explore the predisposing factors and associations of glomerular disease, especially FSGS.

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Data on ethnic differences of lupus nephritis (LN) are extremely scattered in the literature. Furthermore, most of the publications on LN in Arabs have in fact discussed the condition within the general frame of systemic lupus erythematosus (SLE) disease in mixed, but Arab-dominated groups of patients, and fewer, in exclusively Arab individuals. Lupus nephritis however, seems to be common, occurring in 37-69% of SLE patients in the Arab dominated groups and in 50% of the exclusively Arab group. In two out of three groups who reported their renal biopsy results, WHO class III was the most common histopathological type (36% and 54% respectively). Interestingly, the relatively high prevalence of nephropathy in the two groups was associated with a low prevalence of discoid lupus. Arabs with LN who have been studied for serological correlation, did not exhibit any specific or distinctive entity of autoantibody profile. Nonetheless, contradictory data have been noticed in relation to dsDNA antibodies, as they significantly correlated with LN in some of the Arab dominated groups, but not in the uniformly Arab group. Anti­RNP antibodies significantly correlated with non-nephritic SLE patients in the latter group, suggesting a possible protective role of these antibodies. The previously reported correlation between anti-Sm antibodies was not confirmed in the Arab groups. Like the other features of SLE, the differences related to LN in various populations are very likely to be multifactorial rather entirely being ethnical. Lupus nephritis in Arabs however, requires further demarcation by evaluating larger number of patients preferably through multicenter studies.

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Identification of the profile of glomerular disease in a particular geographical region is of fundamental academic, clinical and epidemiological importance. It helps in the recognition of specific risk factors and subsequent planning for adequate prevention. In the present study, 1234 consecutive renal biopsies referred to the nephropathology team of Cairo University over two years were retrospectively analyzed. The main indications for biopsy included nephrotic syndrome, persistent sub-nephrotic proteinuria, recurrent hematuria, suspected secondary hypertension, lupus nephritis and acute and chronic renal failure of undetermined etiology. Proliferative forms of glomerulonephritis [GN] (32.1%) and focal and segmental glomerulosclerosis [FSGS] were the most prevalent lesions among patients with the nephrotic syndrome (22.6%). In subjects with sub­nephrotic proteinuria, FSGS was the principal lesion followed by proliferative lesions. Although all forms of GN were encountered in those presenting with recurrent hematuria, mesangioproliferative GN and FSGS were significantly more frequent. IgA glomerular deposits were detected in 9.8% of all GNs and 15% of those presenting with hematuria. One half of the biopsies obtained for the assessment of suspected secondary hypertension showed only changes compatible with the effect of hypertension per se, i.e. nephroangiosclerosis. On the other hand, a parenchymal renal lesion was found in 52.9% of biopsies. The common glomerular pathologies in patients with lupus nephritis were Classes III and IV. Among patients with chronic renal failure, the predominant lesion was chronic interstitial nephritis (32.6%). An acute interstitial inflammatory element was also detected in 8.4% of cases. About one third of the biopsies obtained for acute renal failure showed acute tubular (11%) or cortical (13.2%) necrosis. Another third showed vasculitis (17.6%) or acute interstitial nephritis (14.3%), and the remaining showed chronic lesions in which the rapid deterioration was probably functional.

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Only few studies regarding glomerulonephritis, with relatively small numbers of patients, have so far been published from different centers in Saudi Arabia, and have reported conflicting results regarding the patterns, even in the same city. The possible reasons for these differences include the small number of patients in the different studies, differences in the indications for renal biopsies, referral bias, geographical differences, and, sometimes, the non­availability of the necessary diagnostic facilities in the reporting centers. In order to overcome these problems, a registry for glomerulonephropathy was attempted in Saudi Arabia. Six large referral hospitals from different regions of Saudi Arabia participated in this registry. Biopsy reports and clinical information of 1294 renal biopsies were obtained. There were 782 renal biopsies due to glomerulonephritis (GN) accounting for 77.2% of the total biopsies. Five hundred eighty seven (72.6%) were primary glomerulonephritidis. Focal and segmental glomerulosclerosis (FSGS) (21.3%) and membrano-proliferative glomerulonephritis (MPGN) (20.7%) were the most common types found in the primary glomerulonephritidis. Membranous glomerulonephritis (MGN) was present in only 10.6% of the cases. IgA nephropathy was found in 6.5% of the cases. Of the secondary glomerulo-nephritides, systemic lupus erythematosus (SLE) was the most common indication for biopsy (57.0%) and amyloidosis was found in only 3.2% of the biopsies. In conclusion, FSGS and MPGN were the most common forms of primary glomerulonephritis in adult patients in Saudi Arabia. MGN was not as common as in the western world. SLE was the commonest cause of secondary GN. Amyloidosis was not as common as in other Arab countries. There is a need for more centers from Saudi Arabia to join this national GN registry. Similar registries can be established in different Arab countries, which all would, hopefully, lead to a Pan­Arab GN registry.

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To evaluate the patterns of glomerular diseases in the Madinah region in Saudi Arabia we reviewed 85 biopsies from native kidneys of adult patients presenting with clinical and or biological features suggestive of glomerular disease. Only light microscopy was used to evaluate these biopsies. There were 62 males and 23 females with age ranging from 12 to 80 years (mean 28 + 13.4 years). The main clinical presenting features were nephrotic syndrome in 68 patients (80%), renal insufficiency in 31 (36.5%), hypertension in 25 (24%) and/or hematuria in 19 (22.4%). Histopathology revealed minimal change disease in 25 patients (29%), mesangioproliferative glomerulonephritis (GN) in 13 (15.3%), focal segmental glomerulosclerosis in 13 (15.3%), membranoproliferative GN in seven (8.2%), membranous GN in three (3.5%), diffuse proliferative GN in five (5.9%), crescentic GN in four (4.7%), lupus nephritis in 13 (15.3%), and amyloidosis in two (2.4%). In conclusion, Minimal change disease was the most common type of primary GN in our patients. The mean age for this group was 27.2 (range 13-43 years). Lupus nephritis was the commonest secondary GN in the Madinah region.

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A prospective study of all native kidney biopsies performed over one year at the Riyadh Armed Forces Hospital, Riyadh, Saudi Arabia was conducted. During this period, 52 kidney biopsies were performed of which, 49 had adequate tissue. All biopsies were processed for light microscopy, immunofluorescence and electron microscopy. The indications for biopsy included the nephrotic syndrome (n=28; 53.8%), asymptomatic proteinuria (n=12; 21.2%), acute nephritic presentation (n=7; 13.5%) and asymptomatic hematuria (n=7; 13.5%). Primary glomerulonephritis (GN), excluding IgA nephropathy (IgAN) was seen in 34 of the 49 patients (77.6%). Focal and segmental glomerulosclerosis and mesangial proliferative GN were the most common histological diagnoses (31% and 20.4% respectively). Surprisingly, we found a high prevalence of IgA nephropathy (IgAN) of 14.5% in comparison with other studies. The prevalence of mesangiocapillary glomerulonephritis (MCGN) was low (2%) and can only be explained as incidental. The study patients were followed-up for an average of 26.3 weeks. At the end of the observation period, 50% has unchanged course, 37.5% had improved their renal function and protein excretion, and 12.5% had deteriorated. The prognosis of different GN groups and renal survival rate cannot be assessed or calculated in this study because of the relatively short duration of follow-up. Our study further emphasizes the need for a national GN registry and long-term follow-up, in order to recognize the common patterns of GN, their natural histories, the appropriate line of management, and to try and arrest their progression to end­stage renal disease.

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In this study, we reviewed the records and the histological findings of a total of 350 adequate native kidney biopsies at both the Jordan University Hospital (260 biopsies in the period 1986-1989) and the Jordan Hospital (90 biopsies in the period 1997-1999). There were 187 males (53.4%) and 163 females (46.6%). Their ages ranged between 12-77 years. The nephrotic syndrome was the presenting feature in 194 (55.4%) patients, acute renal failure in 55 (15.7%), hematuria and/or non-nephrotic proteinuria in 45 (12.9%), chronic renal failure in 32 (9.1%) and hematuria alone 24 (6.9%). In the patients with the nephrotic syndrome, 140 (72.2%) had primary nephrotic syndrome. In this group membranoproliferative glomerulonephritis (MPGN) was the most common pathology and was detected in 49 patients (35%) followed by focal segmental glomerulosclerosis (FSGS) in 38 (27.1%). Amyloidosis and systemic lupus erythematosus were the commonest findings among the 54 patients with secondary nephrotic syndrome (40.7% and 38.8% respectively). Rapidly progressive glomerulonephritis was seen in 34.5% of those patients presenting with acute renal failure, while FSGS was seen in 25% of those with chronic renal failure. Finally IgA nephropathy was the major finding among both the 45 patients with hematuria-proteinuria and the 24 patients with isolated hematuria (26.2% and 66.6% respectively).

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