Plasmapheresis for removal of myeloperoxidase antineutrophil cytoplasmic antibodies: a case report.

We report on a patient with myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) associated glomerulonephritis who had an elevated MPO-ANCA level and necrotizing crescentic glomerulonephritis on renal biopsy. She was treated by double filtration plasmapheresis and immunoadsorption plasmapheresis combined with steroid therapy and immunosuppressive agents. After plasmapheresis, the MPO-ANCA level decreased, and the cellular crescents were reduced. We conclude that plasmapheresis combined with steroid and immunosuppressive therapy may be useful to decrease the activity of MPO-ANCA associated crescentic glomerulonephritis.     

Cytapheresis for the treatment of myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis: report of five cases.

To minimize the adverse effects of high-dose administration of steroids and cyclophosphamide in patients with myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA), granulocytapheresis (GCAP) or leukocytapheresis (LCAP) was performed to reduce inflammation. Four patients with rapidly progressive glomerulonephritis (RPGN) and one patient with pulmonary hemorrhage due to MPO-ANCA-associated vasculitis were treated by cytapheresis. The prednisolone (PSL) dose was 0.28 +/- 0.15 mg/kg/day (mean +/- SD) (range 0.18-0.50 g/kg/day). In the 4 RPGN patients, the peak serum creatinine level was 3.7 +/- 1.9 mg/dl (range 1.7 to 5.6 mg/dl). GCAP was performed in 3 RPGN patients and in 1 pulmonary hemorrhage patient. LCAP was performed in 1 RPGN patient. In the 4 RPGN patients, renal function improved after combined therapy with cytapheresis and corticosteroids. In the pulmonary hemorrhage patient, evidence of pulmonary hemorrhage on chest computed tomography scanning diminished after combined therapy with cytapheresis and corticosteroids. Cytapheresis, when combined with a low-dose or intermediate-dose PSL regimen, is effective in the treatment of ANCA-associated vasculitis.     

Plasmapheresis in antineutrophil cytoplasmic antibody-associated systemic vasculitis.

Small vessel vasculitis syndromes associated with antineutrophil cytoplasmic antibodies frequently cause a necrotizing and crescentic glomerulonephritis with the potential to progress rapidly to permanent renal failure. These conditions are conventionally treated with immunosuppressive drugs, but the possibility that humoral factors are important in their pathogenesis has led to the evaluation of plasmapheresis as an adjunctive therapy. Both controlled and uncontrolled studies have suggested that the routine addition of plasmapheresis is unnecessary. However, when renal function is impaired to the point that dialysis is required, the addition of plasma exchange increases the chance of renal recovery. The superiority of this approach over pulses of methylprednisolone remains to be confirmed.     

The partial recovery of kidney function in chronic uremia patients during hemodialysis treatment

Out of 432 patients placed on the treatment with hemodialysis (HD) for terminal renal failure (TRF) at the All-Union Nephrologic Center from January 1, 1978 to December 31, 1987, 17 patients manifested partial recovery of renal function, which enabled dialysis treatment to be discontinued for a time. Among the 17 patients with noticeable improvement of renal function, 8 presented with lupoid rapid-progressing glomerulonephritis (RPGN), 2 with RPGN associated with hemorrhagic vasculitis, 1 with idiopathic RPGN, 4 with chronic glomerulonephritis (CGN), 1 with chronic pyelonephritis, and 1 with polycystic kidneys. In 11 patients with RPGN, the rate of renal failure progression, expressed by the regression coefficient, was much higher among those in whom HD treatment was discontinued that in the group of patients without renal function recovery. In the 4 patients with CGN, renal function was recovered after the correction of marked disorders of purine metabolism, whereas in the 1 patient with chronic pyelonephritis and in the 1 with polycystic kidneys after urinary infection elimination. According to the ultrasonography data, out of the 17 patients with partial recovery of renal function, the size of the kidneys turned out normal in 14 patients.     

Apheresis for MPO-ANCA-associated RPGN-indications and efficacy: lessons learned from Japan nationwide survey of RPGN

A national survey concerning rapidly progressive glomerulonephritis (RPGN) was conducted in Japan between 1989 and 2000 and resulted in the registration of 715 patients with RPGN. Among the documented patients, the most frequent primary disease was primary pauci-immune crescentic glomerulonephritis (n = 283), and the second most frequent was microscopic polyangitis (n = 127). Overall, 370 patients had MPO-ANCA, and 23 patients had PR3-ANCA. We found that both renal and patient survivals were significantly worse in patients with MPO-ANCA-associated RPGN than patients with PR3-ANCA. Fifty-three patients received apheresis therapy with various combinations of immunosuppressive regimens. They had higher serum creatinine, higher CRP, and a higher frequency of complicated pulmonary involvements as compared to the controls without apheresis therapy. In dialysis-dependent patients, no additional benefit from apheresis therapy was observed. Only pulmonary renal syndrome patients with CRP > 6 mg/dl at presentation showed a slightly better prognosis (patient survival with apheresis; 66.7%, without apheresis; 56.7%). Furthermore, a rapid MPO-ANCA titer reduction was observed in patients treated with apheresis. Patients with MPO-ANCA-associated RPGN were older, and had more chronic and sclerotic lesions than patients with PR3-ANCA-associated RPGN. Based on these findings, we suggest that a lower dose of immunosuppressant should be considered in order to avoid opportunistic infection. In this situation, cytapheresis is the treatment of choice. Nevertheless, in patients with an aggressive form of RPGN with rapid deterioration of renal function like the PR3-ANCA-associated RPGN, or pulmonary renal syndrome complicated severe inflammation, or relapses with high MPO-ANCA titer, we conclude that apheresis therapy should be considered.     

Plasmapheresis in the treatment of rapidly progressing glomerulonephritis

The authors are of the opinion that plasmapheresis (PP) combined with immunosuppressant therapy is an effective and a relatively safe method for the treatment of rapidly progressing glomerulonephritis (RPGN). Introduction of PP in multimodality treatment of RPGN made it possible to arrest rapidly progressing renal failure in all 6 treated patients. After PP treatment was over, renal function was recovered completely in 3 patients. One patient manifested the retention of renal failure of medium degree while rare hemodialysis sessions permitted one to control water-electrolyte disorders. In two patients the discontinuation of PP treatment resulted in the progress of renal failure. The data obtained do not make it possible to relate the improvement of renal function exclusively to the action of PP, since all the patients received immunosuppressants. Nevertheless, in 2 cases, the improvement could be attributed to PP, for its discontinuation in these patients (without any changes in the remaining treatment) brought about again the progress of renal failure.     

Solid tumour and glomerulopathy

We retrospectively examined the prevalence of solid tumours in patients with glomerulonephritis (GN) followed in our regional renal unit between 1977 and 1994. We identified 17 cases of what was thought to be solid-tumour-related glomerulonephritis. Tumours and GN were diagnosed together in six cases, and within a year of each other in another four. In addition, there were seven other cases with a weaker temporal relationship (median duration between GN and cancer diagnosis, two and a half years) but which nonetheless could be tumour-related. In total, there were seven membraneous GN, four mesangial proliferative GN, five crescentic GN and one case of focal segmental GN. Bronchogenic (6) and gastrointestinal carcinoma (CA) (5) were the commonest tumours encountered. Other tumours included breast CA (1), renal cell CA (1), prostatic CA (1), and epithelial thymoma and a leiomyosarcoma of the lung. All MGN and mesangial proliferative GN cases developed nephrotic range proteinuria, whereas all patients with rapidly progressive crescentic GN presented with acute renal failure. Four cases had received immunosuppressive therapy prior to tumour diagnosis. We discuss the validity of each case as tumour-related glomerulonephritis.      

IgA deposits along glomerular basement membranes in rapidly progressive glomerulonephritis

This case presents a rare type of crescentic glomerulonephritis characterized as IgA deposits predominantly along the glomerular basement membranes (GBM). The patient clinically manifested with rapidly progressive glomerulonephritis (RPGN) without pulmonary hemorrhage or vasculitis-related systematic symptoms. No positive results were found on antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), or anti-GBM antibody detection. Therapy with pulse methylprednisolone and intravenous cyclophosphamide was less effective. This case does not belong to the present three categories of crescentic glomerulonephritis based on the clinical characteristics, serum test, immunofluorescence, and electron microscopic findings.     

Focal segmental necrotizing glomerulonephritis in rheumatoid arthritis

We report ten patients with rheumatoid arthritis (RA) who developed a focal segmental necrotizing glomerulonephritis (FSNGN) and extracapillary proliferation typical of vasculitic glomerulonephritis. Five patients also had extrarenal vasculitis. Renal presentation was with renal impairment (n = 9) (median creatinine 726 mumol/l, range 230- 1592 mumol/l), microscopic haematuria (n = 8) and proteinuria (n = 10). Nine patients were seropositive for rheumatoid factor and nine had bone erosions. Serum from four of five patients tested by indirect immunofluorescence was positive for antineutrophil cytoplasmic antibody (ANCA) with perinuclear staining. Only three patients had penicillamine or gold therapy. Treatment was with prednisolone and cyclophosphamide (six patients, two of whom were also plasma-exchanged), prednisolone and azathioprine (two patients) and prednisolone alone (two patients). There was a marked improvement in renal function in eight patients. Two patients with dialysis-dependent renal failure recovered renal function, although in one patient this was transient and she required further dialysis 4 months later. Two other patients progressed to dialysis at 3 months and 1 year respectively. Four patients died, one remains dialysis-dependent, and four continue to have good renal function at 5 year follow-up (median creatinine 148.5 mumol/l, range 120-193 mumol/l). One patient was lost to follow-up at 5 years. FSNGN should be considered in all patients with RA and renal impairment, proteinuria and/or microscopic haematuria. This diagnosis appears to be more likely in patients with clinical extrarenal vasculitis, bone erosions or who are seropositive. In these cases, an urgent renal biopsy is indicated.      

Therapeutic plasma exchange for the treatment of rapidly progressive glomerulonephritis.

Therapeutic plasma exchange (TPE) has been widely accepted as a successful means of removing the antiglomerular basement membrane (anti-GBM) antibodies that result in the rapidly progressive glomerulonephritis (RPGN) of Goodpasture's syndrome. TPE has also been investigated as a means of removing the immune complexes associated with the glomerulonephritides of systemic lupus erythematosus, IgA nephropathy, Henoch Sch?nlein purpura, and cryoglobulinemia. Recently, an antineutrophil cytoplasmic antibody (ANCA) has been implicated in the pathogenesis of RPGN associated with such diseases such as Wegener's granulomatosis and periarteritis nodosa. ANCA has also been found in many cases of RPGN formally considered to be idiopathic. The identification of this autoantibody has given new credence to the possibility that TPE may be beneficial in the treatment of these diseases. This article reviews the data regarding the use of TPE for RPGN.     

Urinary macrophage counts and ratio to T lymphocytes: Possible use in differential diagnosis and management of glomerular disease

The noninvasive method that can differentiate hematuria-positive patients has not yet been developed. We evaluated the clinical value of the analysis of mononuclear cells in urine in combination with urinary erythrocytes as a noninvasive differential diagnostic tool of glomerular disease. The number of macrophages (CD14⁺ cells/ml urine) and T-lymphocytes (CD3⁺ cells/ml urine) were measured by flow cytometry using samples of freshly voided urine from 203 patients with hematuria. They had various types of proliferative glomerular disease, including rapidly progressive glomerulonephritis (RPGN), IgA nephropathy (IgAN), and membranoproliferative glomerulonephritis (MPGN), or nonproliferative glomerulopathy including idiopathic renal hematuria and hereditary nephropathy. Urinary macrophage counts increased significantly with the severity of glomerulonephritis; their number consistently exceeded that of T-lymphocyte counts in patients with active proliferative glomerulonephritis. Urinary macrophage counts in patients with proliferative GN were consistently higher than those of hematuria-matched nonproliferative GN. Moreover, urinary macrophage counts in patients with RPGN were significantly higher than those of MPGN and IgAN. Most of the patients with inactive proliferative glomerulonephritis or with nonproliferative glomerulopathy showed no marked increase in urinary macrophages. Although some patients with nonproliferative glomerulopathy who exhibited gross hematuria showed a slight increase in urinary macrophage counts, such counts were consistently lower than those of T lymphocytes. These observations suggests that urinary macrophage count and its ratio to T-lymphocyte count may provide useful information for clinicians in managing patients with proliferative glomerular disease as well as deciding whether to conduct renal biopsy in patients with hematuria. © 1996 Wiley Liss, Inc.     

Efficacy of plasma exchange in severe idiopathic rapidly progressive glomerulonephritis. A report of ten cases

The vast majority of patients with idiopathic rapidly progressive glomerulonephritis (RPGN) develop irreversible renal failure within weeks to months. We report a retrospective study of 10 patients with idiopathic RPGN who were treated with plasma exchange in addition to steroids and immunosuppression. Renal biopsies were obtained in nine patients. RPGN without immune complexes was present in four, immune complex disease in four, and anti-glomerular basement membrane disease in two. Renal function did not recover in those patients with anti-glomerular basement membrane disease. In contrast, seven of eight patients without auto-antibodies to basement membranes responded to therapy, and four did not need dialysis for two years or more. The sustained improvement in renal function in these seven patients suggests the need for evaluation of plasma exchange as an adjunct to steroids and immunosuppression in a prospective, controlled study in RPGN.     

RENAL DISEASE ASSOCIATED WITH ANTINEUTROPHIL CYTOPLASMIC ANTIBODY: 39 PATIENTS OVER A 5-YEAR PERIOD

SUMMARY We have reviewed 39 adult patients who presented over a 5-year period with biopsy confirmed renal disease in association with positive antineutrophil cytoplasmic antibody (18 with C-ANCA, 21 with P-ANCA). Twenty-three (59%) had primary systemic vasculitis, typically with aggressive renal histology including focal necrotising and crescentic glomerulonephritis. In the remaining patients a wide range of clinical syndromes and renal histological appearances were apparent: 30 had abnormal renal function (serum creatinine >140 μmol/l), including 17 who were initially dialysis-dependent. Intensive immunosuppression was administered in 33 cases. When response was assessed at 3 months, renal function was stable or improved in 17 (52%), 5 of whom were able to discontinue dialysis. There was, however, an appreciable early mortality and, at latest follow-up (1–57 months), 12 patients had died and 8 were on the dialysis programme. On immunosuppression, ANCA became negative in the majority (median time 1.5 months) but recurred during clinical relapse in 11 cases. In asymptomatic patients (12 cases), the reappearance of ANCA positivity did not herald clinical relapse. The ANCA assay has increased awareness of systemic vasculitis but not removed the need for histological confirmation before instituting immunosuppression.     

Plasmapheresis for crescentic IgA nephropathy: a report of two cases and review of the literature

Idiopathic IgA nephropathy is widely regarded as a slowly progressive disease that not infrequently results in end-stage renal failure. Only a minority of patients present with either a rapidly progressive form of glomerulonephritis, or with end-stage renal failure. Anecdotal reports of improved renal function after treatment with plasmapheresis have been published, but the efficacy of this therapy remains controversial. We describe the course of two young males presenting with uremia, hypertension, nephrotic-range proteinuria, and crescentic glomerulonephritis on renal biopsy. Both patients underwent therapy with steroids, immunosuppressive agents, and plasmapheresis without an appreciable improvement in renal function. A review of the literature does not offer any conclusive data to support the role of plasmapheresis in the treatment of rapidly progressive glomerulonephritis due to IgA nephropathy and points out the need to define criteria that may identify subsets of patients with this disorder who may potentially benefit from plasma exchange therapy. J. Clin. Apheresis 14:185-187, 1999. Published 1999 Wiley-Liss, Inc.     

Late onset systemic lupus erythematosus and lupus-like disease in patients with apparent idiopathic glomerulonephritis

We report 17 patients who presented with either apparent idiopathic glomerulonephritis (16 patients) or post-streptococcal glomerulonephritis (one patient). Doubts arose about the nature of these patients' disease, either because their initial renal histology was suggestive of systemic lupus erythematosus (SLE) in the absence of its clinical or serological features, or because they developed with time the clinical or serological features of SLE. Three patients had a positive antinuclear antibody (ANA) test at the onset of their illness, but normal levels of serum binding of double-stranded DNA (dsDNAB). In another four patients the dsDNAB was slightly raised but with a negative ANA. On renal biopsy the predominant appearance was membranous glomerulonephritis (GN) in 10, subendothelial mesangiocapillary GN (MCGN) in three, and focal segmental glomerulosclerosis in two; one patient each had a focal proliferative GN and a diffuse endocapillary GN. On 1 micron renal sections stained with toluidine blue, 10 patients had immune deposits at multiple sites within the glomeruli. Over a period of one to 14 years, six patients developed extrarenal features suggestive of SLE, nine a positive ANA, and 12 increased serum levels of dsDNAB. Five patients became hypocomplementaemic. Cryoglobulins were isolated from the sera of 10 out of 12 patients; seven contained DNA. Separated cryoglobulin IgG from eight patients showed antibody activity directed against both ss and dsDNA in four, and against dsDNA only in three. On the basis of the clinical, histological and serological observation during follow-up five patients were reclassified as definite SLE, four as probable SLE and two as possible SLE. Rarely, SLE may present with nephritis as the sole disease manifestation, antedating other clinical features and even immunological markers of the disease by years. In addition, some patients with a glomerulonephritis may show clinical and immunological, or histological features of SLE, but do not fit accepted definitions of the disease.     

Endocarditis-associated rapidly progressive glomerulonephritis mimicking vasculitis: a diagnostic and treatment challenge

BackgroundInfective endocarditis (IE)-associated rapidly progressive glomerulonephritis (RPGN) is rarely reported. Sporadic case reports have noted the diagnostic and therapeutic challenge in IE-associated glomerulonephritis because it may masquerade as idiopathic vasculitis.MethodsPatients with clinical diagnosis of IE-related RPGN in a tertiary hospital in China between January 2004 and May 2021 were identified and retrospectively reviewed.ResultsTwenty-four patients with IE-associated RPGN were identified. All patients presented with fever and multiorgan system involvement on top of heart and kidneys, spleen (79%, 19/24), skin (63%, 15/24), lung (33%, 8/24) and nervous system (17%, 4/24). Six of the 24 patients (25%) were initially suspected to have ANCA-associated or IgA vasculitis. Forty-five percent of patients are seropositive for ANCA. Renal histology showed mesangial and/or endocapillary hypercellularity with extensive crescents in most patients. C3-dominant deposition was the predominant pattern on immunofluorescence and pauci-immune necrotising crescentic glomerulonephritis was observed in one case. All patients received antibiotics with or without surgery. Six patients received immunosuppressive therapy before antibiotics due to misdiagnosis and seven patients received immunosuppressive therapy after antibiotics due to persistence of renal failure. Three of the 24 patients died due to severe infection. All the surviving patients had partial or complete recovery of renal function.ConclusionIE-associated RPGN is rare and the differential diagnosis from idiopathic vasculitis can be challenging due to overlaps in clinical manifestations, ANCA positivity and absence of typical presentations of IE. The prognosis is generally good if antibiotics and surgery are not delayed. The decision on introducing immunoruppressive treatment should be made carefully on a case by case basis when kidney function does not improve appropriately after proper anti-infective therapy.

Key messages

• Infective endocarditis associated RPGN is rare and differentiating it from idiopathic vasculitis can be challenging due to overlap in clinical manifestations, ANCA positivity and occasional absence of typical manifestations of infective endocarditis.
• Kidney function usually responds to antibiotic therapy alone.
• Immunosuppressive therapy may be beneficial in carefully selected patients whose kidney function does not improve with antibiotics alone.

     

The treatment of ANCA-associated rapidly-progressive glomerulonephritis and Goodpasture syndrome with therapeutic apheresis

Therapeutic plasma aphresis (plasmapheresis) is one form of treatment that is frequently used in practice of Nephrology. Plasmapheresis is the most important part of the therapies for Goodpasture's syndrome and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis which are causes of rapidly progressive glomerulonephritis. The reason why the effectiveness of plasmapheresis therapy cannot be clearly demonstrated in renal involvement in these diseases is that it does not appear to be possible to recruit an adequate number of patients and plasmapheresis is not effective in advanced disease if early treatment is not initiated.     

Glomerulonephritis after ventriculo-atrial shunt

We describe five patients with glomerulonephritis (GN) associatedwith cerebrospinal fluid shunt insertion to relieve hydrocephalus.A ventriculo-atrial (V-A) shunt had been placed on average 12.5years prior to the diagnosis of nephritis (range 0.5–21years). Four patients developed membranoproliferative glomerulonephritis(MPGN) with associated hypocomplementaemia. A single patientdeveloped focal proliferative glomerulonephritis. Coagulasenegativestaphylococci were cultured in four patients, either from bloodor from the shunt. Four patients had their shunts removed, twoof whom also received antibiotics. The other patient receivedantibiotics alone for infective endocarditis due to staphylococcalbacteraemia which originated in the shunt. All patients hadsubstantial renal impairment at the time of diagnosis (GFR,glomerular filtration rate, 20–45 ml/min). There was significantimprovement in renal function after appropriate treatment; fourof the five patients doubled their GFRs and two patients regainednormal function.     

ANCA-associated vasculitis--an autopsy study

The purpose of the study was to analyse the autopsies of 31 patients (20 female, mean age 67 years, range 28-87 years; and 11 male, mean age 66 years, range 47-80 years) with antineutrophil cytoplasmic antibodies (ANCA)-positive vasculitis, which was clinically confirmed in 25 patients and suspected in 6 patients, who had been treated and had died between 1989 and 1999. Kidney biopsy was performed in 22 patients on average 33 months (range, 1-132 months) prior to death. Biopsy and autopsy tissue specimens were examined by standard light and immunofluorescence microscopy techniques. Pauci-immune extracapillary glomerulonephritis was found in nearly the same percentage of 22 renal biopsies and 31 autopsies, namely in 91% and 84%, respectively. Active necrotising extracapillary glomerulonephritis was the prevailing lesion in 75% of biopsies, while advanced sclerosing glomerular lesions prevailed in 69% of autopsies. In the biopsies, necrotising lesions predominated in patients with ANCA of proteinase 3 specificity, while sclerotic lesions were more often associated with myeloperoxidase-ANCA. In the autopsies, florid necrotising systemic vasculitis coexisted in 2 patients with advanced sclerosing glomerulonephritis. Autopsies revealed the actual expansion of vasculitic disease, disclosed clinically silent vasculitic involvement of unusual locations and, in 3 patients, confirmed the clinically suspected vasculitis. The final diagnoses in 31 patients were as follows: Wegener's granulomatosis (5 men, 2 women), microscopic polyangitis (10 women, 2 men), pauci-immune crescentic glomerulonephritis (4 women, 3 men), a single case each of polyarteritis nodosa and isolated cutaneous vasculitis. In 3 patients, suspected vasculitis was not confirmed at autopsy. Nineteen of 31 patients died from septic infections or necrotising pneumonias, and 6 patients from progressive or recurrent vasculitis with complications, altogether nearly 80%. Cardiovascular failure, including pulmonary thrombembolism, caused death in 6 patients.     

Correlation among WHO classes, histomorphologic patterns of glomerulonephritis and glomerular immune deposits in SLE

In addition to the conventional World Health Organization (WHO) classification of lupus glomerulonephritis (GN), various concomitant approaches have been introduced in the evaluation of renal biopsies of patients with systemic lupus erythematosus (SLE) in order to increase the impact of biopsies on the decision concerning the most appropriate therapy as well as for establishing the prognosis. Three hundred and seventy kidney tissue samples from 267 SLE patients were analysed using standardised light, electron and immunofluorescence microscopic techniques. In 155 patients, a comparative clinical follow-up study and statistical analysis were performed. The study highlighted the heterogeneity of WHO classes IV and III, which include 5 and 6 different conventional histomorphologic types of GN, respectively. Mixed membranous and proliferative GN associated with "full-house" mesangial-transmembranous immune deposits, demonstrated in more than one third of our SLE cases, appears to be diagnostically most characteristic. Immune deposits distributed in the glomeruli in five different patterns, obviously play a major role in the pathogenesis of various WHO classes and histomorphologic types of lupus GN. Additional mechanisms related to the occurrence of antiphospholipid antibodies and antineutrophil cytoplasmic antibodies are suggested to contribute to the histomorphologic heterogeneity of WHO class III and IV lupus GN, particularly to the development of thrombotic, necrotising and crescentic glomerular lesions. In the present study, a statistically significant association was demonstrated between increasing mean values of the activity index and glomerular deposit distribution patterns labeled by subendothelial deposits. Furthermore, a significant correlation was established between an increasing risk of developing renal failure and increasing mean values of the chronicity index. Differences in the increasing risk of developing renal failure between groups with different histomorphologic types of GN and different immune deposit distribution patterns were not statistically significant. The surprisingly high renal survival rate of more than 80% noted in lupus patients with predominantly necrotising crescentic GN during the mean follow-up period of 40 months appears to be related to the more aggressive treatment of those patients. Our study confirmed a significant role of the WHO classification of lupus GN in the decision concerning the most appropriate treatment and prognostication. An increasing risk of irreversible renal failure in patients with WHO class IV lesions in contrast to those of WHO class III and in contrast to those of the category incorporating all other WHO classes was shown to be statistically significant.