Heterogeneity of Prognosis in Adult IgA Nephropathy, Especially

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1 ORIGINAL ARTICLE D Heterogeneity of Prognosis in Adult IgA Nephropathy, Especially with Mild Proteinuria or Mild Histological Features Joichi Usui, Kunihiro Yamagata, Hirayasu Kai, Takaaki Outeki, Satoshi Yamamoto, Kaori Muro, Aki Hirayama, Keigyo Yoh, Chie Tomida**, Kouichi Hirayama, Satoshi Suzuki** *, Masaki Kobayashi****, Michio Nagata* and Akio Koyama Abstract Objective The present study was undertaken to clarify the clinical course and prognosis of adult patients with primary IgA nephropathy (IgAN), especially with mild proteinuria or mild histological alternations. Patients and Methods A population of 735 IgAN patients whomwe were able to observe for morethan two years was examined. Results A total of 115 patients (15.6%) was on dialysis during the observation period. The overall 5-year renal survival rate was 92.0%. On the other hand, 166 patients (22.6%) were in clinical remission. A group with mild proteinuria included 197 patients (26.8 % ). Forty-seven patients of this group showedminor glomerular abnormalities, whereas 12 patients with mild proteinuria showed severe mesangial involvement. Three patients with mild proteinuria were on dialysis during the observation period, whose proteinuria was increased during the clinical course. A group with minor glomerular abnormalities included 82 patients (1 1.2 % ). Forty-seven patients of this group showed mild proteinuria, of whom12 patients showed moderate proteinuria. However, three patients with minor glomerular abnormalities who were not on dialysis showed loss of renal function. Conclusion These results indicated the heterogeneity of the course and prognosis in IgAN. Even if a patient's initial clinical or histological findings are comparatively mild, strict follow-up management is needed. (Internal Medicine 40: , 2001) Key words: clinical remission, minor glomerular abnormalities Introduction Thirty-two years has passed since IgAN was first described by Berger and Hinglais (1). Thus finally we are at a point where we can elucidate the long-term clinical features of IgAN, including its course and prognosis. According to the latest investigations, progression to end stage renal disease (ESRD) occurred in approximately 30% of cases over a 20-year period, and IgANwas ascertained to be a disease with a poor prognosis (2-8). On the other hand, clinical remission of this disease has often been reported, so IgANis also knownto be a disease with heterogeneous prognosis (5, 9-1 1). Clinical and histological risk factors known to predict a poor prognosis have been cited as targets of investigations at diagnosis. However, IgAN follows a waxing and waning course, and it is nearly impossible to predict poor prognosis by clinical and histological findings at any specific time except in advanced cases. In the present study, we evaluated the prognosis of IgAN patients, focusing particularly on mild clinical and histological findings at diagnosis, and sought to elucidate the characteristics of cases which progress to ESRD. For editorial comment, See p 679. Patients and Methods Pa tien ts Patients from three institutes, National Sakura Hospital, Hitachi General Hospital, and University of Tsukuba were investigated in this study over a 21-year period from We were able to observe 735 adults diagnosed with primary IgAN by renal biopsy for more than two years after diagnosis. Clinical classifica tion Several parameters were evaluated: amount of urinary pro- From the Department of Internal Medicine, *the Department of Pathology, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, **the Department of Nephrology, Hitachi General Hospital, Hitachi, ***the Department of Internal Medicine, National Sakura Hospital, Sakura and ****the Department of Nephrology, Tokyo Medical University Kasumigaura Hospital, Ami Received for publication August 3 1, 2000; Accepted for publication February 19, 2001 Reprint requests should be addressed to Dr. Kunihiro Yamagata, the Department of Internal Medicine, Institute of Clinical Medicine, University of Tsukuba, Tennodai, Tsukuba, Ibaraki

2 Usui et al tein excretion (UP), serum creatinine value (s-cre), and creatinine clearance (Ccr). Proteinuria was classified as mild if the 24-hour urinary protein excretion was less than 0.5 g, mild to moderate if it was between 0.5 and 0.9 g, moderate if it was between 1.0 and 3.4 g, and heavy if in the nephrotic range of 3.5 g or more. Final renal outcome included the following six states: 1) Clinical remission: normal renal function without urinary abnormalities. 2) Only microscopic hematuria: number of red blood cells in urinary sediment was found above six per high- power field and 24-hour urine protein excretion was less than 0.3 g. 3) Normal renal function with proteinuria: regardless of existence of hematuria. 4) Chronic renal insufficiency (CRI): serum creatinine value above 1.5 mg/dl and regardless of a variety of urinary abnormalities. 5) Chronic renal failure (CRF): serum creatinine value above 2.0 mg/dl and regardless of a variety of urinary abnormalities. 6) End stage renal disease (ESRD): patient on dialysis. Histological classification The renal biopsy specimens in 735 patients were examined at each institute. Both light microscopic and immunofluorescence microscopic findings were available for all patients. In immunofluorescence microscopic findings, glomerulonephritis with predominant IgA mesangial deposits was defined as IgAN. Sufficient material for histological examination (five or more glomeruli including sclerosed ones) was available for 630 of the 735 patients selected on the basis of immunofluorescence findings. The light microscopic findings were classified based on the histological classification of the World Health Organization and included minor glomerular abnormalities (Minor), focal proliferative glomerulonephritis [FPGN; a proliferation involving some but not all (<80%) glomeruli excluding sclerosed glomeruli], diffuse proliferative GN[DPGN; a proliferation involving all or nearly all (>80%) glomeruli excluding sclerosed glomeruli], and sclerosing GN. Patients with insufficient material for renal biopsy (fewer than five glomeruli) and pathological information were defined as the unclassified group. Sta tistical analysis The renal survival rates were calculated by the Kaplan-Meier method, and significant differences in the renal survival rates were evaluated by the generalized Wilcoxon method (univariate analysis). Significant differences in renal survival probability were evaluated by the %2method. Significant differences in clinical parameters were evaluated by Fisher's Protected Least Significant Difference (Fisher's PLSD). A p value of less than 0.05 was considered statistically significant. Clinical characteristics Results There were 394 males and 341 females. Mean age at the time of renal biopsy was 36.5±12.9 years (range, years). Theaverage period of observation from renal biopsy to the end of follow-up was 6.7±4.1 years (range, years). All patients had some urinary abnormalities at diagnosis. The amount of urinary protein excretion at the time of renal biopsy was 1.20±1.34 g (range, 0-10 g), the serum creatinine value was 1.01±0.42 mg/dl (range, mg/dl), and creatinine clearance was 91.7±30.5 ml/min (range, ml/min). Renal outcome The renal outcome of all 735 adult patients with IgANis shown at the end of the observation period (Table 1). Renal function deteriorated in a total of 199 patients, 27.1% of the study population (serum creatinine value above 1.5 mg/dl). Out of them, 115 patients (15.6%) were on dialysis. And 361 patients (49. 1 %), including only microscopic hematuria and only proteinuria groups, had normal renal function with urinary abnormalities. On the other hand, 166 patients, 22.6% of the study population, were in clinical remission. The overall 5-year renal survival rate was 92.0%, and the 10-year renal survival rate was 76.4% (Fig. 1). Proteinuria and prognosis In the present study, 197 patients, 26.8% of the study popu- Clinical remission Table 1. Renal Outcome Outcomes (%) Only microscopic hematuria Normal renal function with proteinuria Chronic renal Chronic renal End stage renal disease Others Total H6 insufficiency failure ll Duration (years) Figure 1. Renal survival rates of 735 adult patients with IgAN. The overall 5-year renal survival rate was 0.920, and the 10- year rate was

3 Heterogeneity of Prognosis in Adult IgAN lation, had mild proteinuria (Table 2). From the histological findings of these patients with mild proteinuria, 47 patients, 23.8% of 197 patients, had minor glomerular abnormalities. On the other hand, 12 (6.1%) of 197 patients, had severe mesangial involvement, including DPGNand sclerosing GN. The clinical parameters were evaluated amongeach group (Table 2). The age was significantly higher as the amount of urinary protein excretion increased [excluding the difference between mild to moderate and heavy groups (p=0. 107), moderate and heavy ones (p=0.959)]. The serum creatinine value was significantly higher and creatinine clearance was significantly lower with the increase in the amount of urinary protein excretion. The number of patients on dialysis at the end of the observation period was shown in each group of patients as classified by the level of proteinuria (Table 3). As the level of proteinuria increased, so did the probability of renal death. Seven patients with mild proteinuria showedloss of renal function (serum creatinine value above 1.5 mg/dl). Three of them, 1.5% of 197 patients, were on dialysis (Table 3). During the observation period, increments in the amountof urinary protein excretion were observed in these patients (data not shown). And the deterioration of renal function was correlated with the increments of proteinuria during the clinical course. One of these patients (Case 1 ) showed only microscopic hematuria at diagnosis (Table 4), and only this case was on dialysis. Twoof these patients (Case 1, 2) revealed at least one of three clinical and histological deteriorating factors, hypertension, deterioration of renal Table 2. Clinical Characteristics of Patients in Each Group Classified by Proteinuria Level Significant differences in age, UP, s-cre and Ccr were evaluated by the Fisher's PLSDmethod. *p<0.01 between each group [excluding the difference between the mild to moderate and heavy groups (p=0.107), moderate and heavy ones (p=0.959)]. **p<0.01 between each group. Table 3. Number of Patients on Dialysis in Each Group as Classified by Proteinuria Level Significant differences were evaluated by the %2method. *p<0.01 between each group. Table 4. Profile of Patients on Dialysis in the Mild Proteinuria Group aht: administration of anti-hypertensive drugs at diagnosis. bthe number of glomeruli in renal biopsy specimens. ctherapy: S : gluco-corticoid, IS: other immunosuppressive drugs, AP: anti-platelet drugs, AC: anti-coagulant, duration between renal biopsy and introduction of dialysis. 699

4 LE Usui et al function and/or sclerosing GN. The 10-year renal survival rates were 94.8% for patients with mild proteinuria, 85.7% for patients with mild to moderate proteinuria, 66.0% for patients with moderate proteinuria and 37.4% for those with heavy proteinuria. There were significant differences in the renal survival based on proteinuria level (Fig. 2). Histology and prognosis Histologically 82 patients, 1 1.2% of the study population, showed minor glomerular abnormalities (Table 5). Within this group with minor glomerular abnormalities, 47 (57.3%) of 82 patients had mild proteinuria. On the other hand, 12 (14.6%) of 82 patients had moderate proteinuria outside of the nephrotic range v; " ^ V, V. Mild &----VMild A... to moderate Moderate ~ Heavy Duration (years) Figure 2. Renal survival rate based on proteinuria level. For patients with mild proteinuria, the 5- and 10-year renal survival rates were 1.00 and For mild to moderate proteinuria, these rates were and For moderate proteinuria, they were and 0.660, and for heavy proteinuria, and The statistical analysis revealed p<0.01 between each group [excluding the difference between the mild and mild to moderate groups (p=0.0142)]. The clinical parameters were evaluated amongeach group (Table 5). The age of the sclerosing GNgroup was significantly higher than in the other three groups, but there was no significant difference between the other groups. The amount of urinary protein excretion was significantly higher with the progression of histological classification [excluding the difference between DPGN and sclerosing GN groups (p=0.078)]. The serum creatinine value was significantly higher [excluding the difference between DPGNand sclerosing GNgroups (p=0.055)] and creatinine clearance was significantly lower [excluding the difference between Minor and FPGNgroups (p=0.052)] with the progression of histological grade. The numberof patients on dialysis at the end of the observation period was examined in each histological classification (Table 6). The probability of exhibiting renal death increased with the progression of histological classification, and the renal outcome was significantly worse in each successively deteriorating histological classification, with the exception of the DPGNand sclerosing GN groups. However, although three patients with minor glomerular abnormalities showedloss of renal function, none of these patients was on dialysis. The 10- year renal survival rates were 100%for patients with minor glomerular abnormalities, 78. 1% for patients with FPGN, 66.1% for patients with DPGNand 15.5% for those with sclerosing GN. There were significant differences in renal survival based on histological classification (Fig. 3). Discussion IgAN is the most commonform of glomerulonephritis, accounting for 10 to 50 % of the cases of primary glomerular diseases in many countries (2, 3, 8). The natural course of IgAN varies considerably. Somepatients run a favorable clinical course for a long time, while others showa slow or rapid loss of renal function, often exhibiting ESRD. The overall percentage of renal death and 5-year renal survival rates in the present study was in accordance with previous reports (2-5, 7, 12), or was better (8). But the 10-year renal survival rate lacked unity. The present result was worse compared with previous reports Table 5. Clinical Characteristics of Patients in Each Histological Group Significant differences in age, UP, s-cre and Ccr were evaluated by the Fischer's PLSDmethod. *p<0.01 between the sclerosing GNand the other 3 groups. **p<0.01 between each group [excluding the difference between DPGNand sclerosing GNgroups (p=0.078)]. #p<0.05 between each group [excluding the difference between DPGNand sclerosing GN groups (p=0.0546)]. ^p^.os between each group [excluding the difference between Minor and FPGNgroups (p=0.0524)]. 700

5 Heterogeneity of Prognosis in Adult IgAN Table 6. Number of Patients on Dialysis in Each Histological Group Significant differences were evaluated by the %2method. *The conclusion was p<0.01 between each group [excluding the difference between DPGNand sclerosing GNgroups (**p=0.632)]. 2 8" 6^^"Iq..FPGN " 4^ * DPGN^jh ^ «0.2- ^ n Sclerosing GNi 0j Duration (years) Figure 3. Renal survival rate based on histology. For patients with minor glomerular abnormalities, 10-year renal survival rate was For patients with FPGN, 5- and 10-year renal survival rates were and For patients with DPGN,these rates were and 0.661, and for patients with sclerosing GN, and The statistical analysis revealed p<0.01 between each group [excluding the difference between DPGNand sclerosing GN groups (p=0.0393)]. (2-5), or resembled them (8, 12). On the other hand, the probability of clinical remission was somewhathigher than the rates cited in previous reports, which ranged from 0 to 23% (5, 9-1 1). In the present study, among the patients with remission, 48 patients, 6.5% of the study population, did not receive commontherapy or administration of immunosuppressive drugs. Taking these findings together, we can suggest that IgANis heterogeneous in clinical course and prognosis. Previously we investigated the histological changes in eight patients of this clinical.remission group by serial renal biopsy (13). According to our results, the histological findings did not improve and renal involvements in both the acute and chronic phases did not change. Costa et al reported improvements in histological findings and a disappearance of IgA deposits in three of four patients with clinical remission on serial renal biopsy ( 10). However, Alamartine et al reported that histological findings of patients with clinical remission did not change before or after the remission (1 1). This latter view is similar to ours. Generally, the clinical findings associated with increased likelihood of progression in many of the reports include older age at onset (4, 5, 14, 15), younger age at biopsy (7), male gender (4, 14), lack of episodic macroscopic hematuria (4-6, 16), presence of microscopic hematuria (14, 16), heavy proteinuria (2-8, 12, 14-20), hypertension (3, 4, 6-8, 14, 15, 16), and renal dysfunction at renal biopsy (2-7, 12, 16, 17, 20). Histological findings associated with a poor prognosis include severe histological grade (2-4, 7, 8, 17, 20, 21), mesangial proliferation (2, 4, 8, 15), glomerular sclerosis (3-6, 15, 19), endocapillary proliferation (3, 19), segmental lesion with or without crescents (4, 5, 12, 14), interstitial nephritis or fibrosis (3-7, 12, 15, 20), hyaline arteriolosclerosis (4, 5, 7), capillary immune deposits (5, 15, 19), and vascular deposits ofc3 (ll). Whenthese obvious clinical or histological findings can be confirmed, the prediction of poor prognosis is comparatively easy. On the other hand, it is also easy to determine the prognosis of most patients whowere judged to have a milder proteinuria and histological findings. However, it was often found that there weresomepatients whohad an unfavorable outcome in spite of these milder clinical and histological findings. Above all, wethought that it was important to distinguish someunfavorable outcomes from the manyfavorable outcomes. Our first endeavor in the present study was to examine the prognosis of patients with mild proteinuria. When proteinuria was mentioned as a risk factor, the standard value of proteinuria usedtobe 1 gperday ormore (4, 6-8, 12, 14, 18, 19). Inthis study, we separated further detailed groups as classified by proteinuria level. This group with mild proteinuria included many patients with minor glomerular abnormalities. However,patients with severe mesangial involvement, including DPGNand sclerosing GN, were also in existence. Most of the patients with severe histological features in the mild proteinuria group did not reach ESRDby the end of the observation period. This may have been due to the aggressive introduction of therapy, mainly administration of gluco-corticoid, to manage the se- 701

6 Usui et al vere histological involvement. In the patients exhibiting ESRDof the mild proteinuria group, the deterioration of renal function was correlated with the increments of proteinuria during the clinical course. It has been generally reported that massive proteinuria is the most important risk factor in determining the progression of IgAN (2-5, 6-8, 12, 14-20), and one report suggested that increased proteinuria during the clinical course is also an obviousrisk factor (17). In the present study, the main cause of the deterioration of renal function in three patients was unknownprecisely because they did not undergo serial renal biopsy during the period of progression. In previous reports, the progression of glomerulonephritis by itself, complications of interstitial and/ or vascular involvements were suspected as the cause for loss of renal function (ll, 14). During the follow-up of the IgAN patients, increased proteinuria is thought to be one of the signs of deteriorating renal function. Then renal biopsy or intensive treatment should be performed even if the initial histological features have been minor glomerular abnormalities. Our next endeavor in the present study was to examinethe prognosis of patients with minor glomerular abnormalities. This group included numerous patients with mild proteinuria, as expected. Conversely, comparative numbers of patients with moderate proteinuria were also included. Surprisingly, no patient in this group reached ESRDat the end of the observation period. This fortunate result is very important, and in particular it may be said that a patient with both mild proteinuria and minor glomerular abnormalities does not risk progression. Actually, three patients with mild histological features showed loss of renal function. They had accompanying moderate proteinuria at diagnosis. But we need further follow-up management of these patients, because the limited amount of specimens obtained by needle renal biopsy mayhave contributed to the unexpected results as including only mild histological involvement. In IgANpatients, clinical and histological features at the initial renal biopsy are closely linked to the prognosis. However, even if their initial findings are comparatively mild, the heterogeneity of the course and prognosis in IgANshould be considered and strict follow-up managementis needed. Acknowledgements: This work was supported in part by a Health Science Research Grant from Ministry of Health, Labour and Welfare, Japan. Parts of this study were presented at the 97th Annual Scientific Meeting of the Japanese Society of Internal Medicine, April 7, 2000, Kyoto. Refere nces 1 ) Berger J, Hinglais N. Les depots intercapillaires d'iga-igg. J Urol Nephrol (Paris) 74: , ) KoyamaA, Igarashi M, Kobayashi M, and Members and Coworkers of the Research Group on Progressive Renal Diseases. Natural history and risk factors for immunoglobulin A nephropathy in Japan. AmJ Kidney Dis 29: , ) D'Amico G, Imbasciati E, Barbiano di Belgioioso G, et al. Idiopathic IgA mesangial nephropathy. Clinical and histological study of 374 patients. Medicine (Baltimore) 64: 49-60, ) D'Amico G. Influence of clinical and histological features on actuarial renal survival in adult patients with idiopathic IgA nephropathy, membranous nephropathy, and membranoproliferative glomerulonephritis: Survey of the recent literature. AmJ Kidney Dis 20: , ) Ibels LS, Gyory AZ. IgA nephropathy: analysis of the natural history, important factors in the progression of renal disease, and a review of the literature. Medicine (Baltimore) 73: , ) Beukhof JR, Kardaun O, Schaafsma W, et al. Toward individual prognosis of IgA nephropathy. Kidney Int 29: , ) Radford MGJr, Donadio JV Jr, Bergstralh EJ, Grande JR Predicting renal outcome in IgA nephropathy. J AmSoc Nephrol 8: , ) Haas M. Histologic subclassfication of IgA nephropathy: a clinicopathologic study of 244 cases. AmJ Kidney Dis 29: , ) Kobayashi Y, Hiki Y, Fujii K, Wakabayashi Y, Tateno S, Chen XM. IgA nephropathy with clinical remission. Nippon Jinzo Gakkai Shi (Jpn J Nephrol) 27: , 1985 (in Japanese). 10) Costa RS, Droz D, Noel LH. Long-standing spontaneous clinical remission and glomerular improvement in primary IgA nephropathy (Berger's disease). Am J Nephrol 7: , ) Alamartine E, Sabatier JC, Berthoux FC. Comparison of pathological lesions on repeated renal biopsies in 73 patients with primary IgA glomerulonephritis: value of quantitative scoring and approach to final prognosis. Clin Nephrol 34: 45-51, ) Katafuchi R, Oh Y, Hori K, et al. An important role of glomerular segmental lesions on progression of IgA nephropathy: a multivariate analysis. Clin Nephrol 41: , ) Usui J, Tomida C, Yamagata K, et al. Investigations of clinical remission cases in adults with IgA nephropathy. Nippon Jinzo Gakkai Shi (Jpn J Nephrol) 41: 319, 1999 (Abstract in Japanese). 14) Nicholls KM, Fairley KF, Dowling JP, Kincaid-Smith P. The clinical course of mesangial IgA associated nephropathy in adults. Q J Med53: , ) Kobayashi Y, Tateno S, Hiki Y, Shigematsu H. IgA nephropathy: prognostic significance of proteinuria and histological alterations. Nephron 34: , ) BeukhofJR, Kardaun O, Ockhuizen Th, van der Hem GK. Kidney survival in IgA nephropathy: multiple regression analysis of genetically differing subpopulations -is IgA nephropathy a real disease entity? Semin Nephrol 7: , ) Kobayashi Y, Kokubo T, Horii A, Hiki Y, Tateno S. Prognostic prediction of long-term clinical courses in individual IgA nephropathy patients. Nephrology 3: 35^0, ) Neelakantappa K, Gallo GR, Baldwin DS. Proteinuria in IgA nephropathy. Kidney Int 33: , ) D'Amico G, Minetti L, Ponticelli C, et al. Prognostic indicators in idiopathic IgA mesangial nephropathy. Q J Med 69: , ) Vleming LJ, de Fijter JW, Westendorp RGJ, Daha MR, Bruijn JA, van Es LA. Histomorphometric correlates of renal failure in IgA nephropathy. Clin Nephrol 49: , ) Lee SMK, Rao VM, Franklin WA, et al. IgA nephropathy: morphologic predictors of progressive renal disease. HumPathol 13: ,

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