Prognostic factors in Wegener's granulomatosis.

We analysed data from 64 patients with Wegener's granulomatosis to determine predictor variables of outcome. The mean period of observation after the diagnosis had been established was 3.2 (range 0.1-11.2) years. At the time of diagnosis, 15 (23%) patients had only local symptoms. The disease was generalized to multiple organs in 49 (77%) patients. Renal biopsies were obtained in 33 patients; 13 (39%) had extracapillary glomerulonephritis, which was the most common renal lesion. All but three patients received immunosuppressive therapy. At time of follow-up, 17 (27%) patients were in complete, and 26 (40%) in partial remission. We employed a Kaplan Meier analysis to identify predictor variables of outcome. Renal involvement, initial creatinine concentration, serum albumin or total protein concentration, leukocyte count and erythrocyturia proved to be predictor variables. These variables may be of value in guiding the intensity of treatment in patients with Wegener's granulomatosis.

[Therapy of Wegener's granulomatosis. Experiences with conventional and stage-adjusted treatment in 111 patients over 24 years]. / Die Therapie der Wegenerschen Granulomatose. Erfahrungen mit der konventionellen und der stadienadaptierten Behandlung bei 111 Patienten während 24 Jahren.

Treatment and outcome of 111 patients who fell ill with Wegener's granulomatosis (WG) between 1966 and 1990 were analysed retrospectively. The mean observation time after diagnosis had been 47 (1-288) months. At the time of diagnosis 37 patients suffered from locoregional symptoms only, whereas the disease was generalised in 74 patients. Two regimes of treatment were differentiated: "conventional" treatment, i.e. daily application of cyclophosphamide/prednisolone (FAUCI scheme) or azathioprine/prednisolone or prednisolone alone, and "stage-adapted" treatment, characterised by change of different treatments (e.g. cyclophosphamide pulse therapy, cotrimoxazole) according to the extent and activity of disease. In patients who received stage-adapted treatment, relapses occurred significantly more frequently than in conventionally treated patients. On the other hand, lethal outcome was much more frequent in conventionally treated patients.

Treatment of Wegener's granulomatosis.

Treatment and outcome of 111 patients who fell ill with Wegener's granulomatosis (WG) between 1966 and 1990 were analysed retrospectively. Two regimens of treatment were distinguished: "conventional" treatment, i.e. daily application of cyclophosphamide/corticosteroids (FAUCI scheme) or azathioprine/corticosteroids or corticosteroids alone, and "stage-adapted" treatment, characterized by change of different treatments (e.g. cyclophosphamide pulse therapy, cotrimoxazole) according to the extent and activity of disease. In patients who received stage-adapted treatment, exacerbations occurred significantly more frequent than in conventionally treated patients, while lethal outcome was much more frequent in conventionally treated patients.

[Wegener's granulomatosis and anti-cytoplasm antibodies]. / Wegenersche Granulomatose und antizytoplasmatische Antikörper.

20 patients with biopsy-proven Wegener's granulomatosis (WG) and 95 control patients underwent determination of anticytoplasmic antibodies (ACPA) by the indirect immunofluorescence technique to assess the specificity and sensitivity of ACPA for WG. Of 14 untreated patients with WG, 13 were ACPA-positive. All these patients became ACPA-negative under immunosuppressive treatment. 4 patients treated with immunosuppressive drugs and 2 patients in remission after termination of therapy had a negative ACPA-test. ACPA were detected in 9 patients of the control group (two patients with Henoch Schoenlein purpura, two patients with systemic vasculitis, 2 patients with systemic diseases, and 1 patient with systemic lupus erythematodes). With that we achieved a specificity of 90.5% and a sensitivity 65.0%. In conclusion the ACPA-determination is very helpful for diagnosis and follow-up of WG.

[Differentiation of hematuria by phase contrast microscopy studies of urine erythrocytes]. / Die Differenzierung der Hämaturie durch phasenkontrastmikroskopische Untersuchungen von Harnerythrozyten.

The results of phase-contrast microscopic investigations of urinary erythrocytes in 300 patients (120 patients with bioptical verified glomerulonephritis, 54 patients suffering from interstitial nephritis, 56 patients with urinary tract diseases and 70 healthy probands) are discussed. The portion of glomerular erythrocytes is dependent on diuresis, renal function and activity of glomerulonephritis. This non-invasive procedure is quick and easy practicable and its results are reliable (sensitivity and specifity of more than 90%). The proof of mainly glomerular erythrocytes save from instrumental and invasive diagnostic procedures in nephrological diseases, whereas non-glomerular erythrocytes may have an urological or hematological origin.

[THe effect of pregnancy on the long-term course of chronic glomerulonephritis]. / Einfluss einer Schwangerschaft auf den Langzeitverlauf der chronischen Glomerulonephritis.

During a long-term study in 34 out of 154 women with bioptically ascertained glomerulonephritis 38 pregnancies were observed. The clinical manifestation took place three times during and thirteen times immediately after the end of the pregnancy. Apart from membranoproliferative glomerulonephritides all morphological forms of glomerulonephritis were present. A nephrotic syndrome was existing in 14 women (41.2%). The patients with pregnancy after on an average 11.3 years observation time showed a stabile renal function in 85.3% in contrast to 71.9% of the other female patients (not significant). The pregnancy, including a nephrotic syndrome, does not deteriorate the long-term prognosis of the glomerulonephritis.

[Cellular immune response and glomerulonephritis--their detection with the Tetamun intracutaneous test]. / Zellgebundene Immunantwort und Glomerulonephritis--ihre Erfassung mit dem Tetamun-Intrakutantest.

In the considerations of the pathogenesis of glomerulonephritis more importance is attributed to cellular immune reactions. The cell-mediated immune response was examined by means of the Tetamun intracutaneous test in 37 patients with histological verified glomerulonephritis, in 18 patients with non-glomerular renal diseases, and in 25 healthy probands. The influence of main disease, renal insufficiency, nephrotic syndrome and immunosuppressive therapy on the test results could be determined by proper group formation. A significant diminution of cell-mediated immune reactions by the glomerulonephritis was verified. The importance of this finding in the pathogenetic routes and a possible model are discussed.

[Glomerulonephritis in sarcoidosis]. / Glomerulonephritis bei Sarkoidose.

The renal involvement in sarcoidosis can be demonstrated as nephrocalcinosis, nephrolithiasis, granulomatous nephritis, and glomerulonephritis. The clinical signs of a renal manifestation are inferior than the morphological findings, so that the reported frequency of 9-25% is an approximate value. In the literature the glomerular changes are rare. In the case of glomerulonephritis the membranous form is dominating; an extracapillar-proliferative glomerulonephritis is described in only 3 cases in the literature. The course of a 16-year-old male patient with an extracapillar-proliferative glomerulonephritis and M. Boeck is described in detail. After a prednisolone therapy for 2 years, which was introduced by methylprednisolone at 1000 mg for 3 days, the renal function was improved continuously.

[Results of a multicenter study of the early detection of glomerulonephritis. 1]. / Ergebnisse einer multizentrischen Studie zur Früherkennung der Glomerulonephritis. 1. Mitteilung.

In the framework of a multicentric retrospective study between selected clinics of the Republic Austria and the GDR anamnestic, clinical and paraclinical data were investigated in their valency for the early recognition of glomerulonephritis. Data of 583 patients were evaluated. Hereby it was shown that independent of the size of excretion and the reproducibility the findings "proteinuria" are of particular significance for the early recognition. The serological investigations usually performed within the diagnostic of glomerulonephritis proved as insignificant for the early recognition. Since the establishing of an exact diagnosis is up to now possible only with the help of invasive methods, a call on research is made to develop reliable, non-invasive diagnostic methods.

[Results of immunosuppressive therapy of chronic glomerulonephritis in 400 patients]. / Ergebnisse der immunsuppressiven Therapie der chronischen Glomerulonephritis bei 400 Patienten.

For the judgment of the success of the immunosuppressive therapy of chronic glomerulonephritis 400 patients (242 males and 158 females) at an average age of 31.2 years were examined after an average duration of treatment of 31.9 months. Apart from the renal function (serum creatinine), the histological and immunohistological form of the glomerulonephritis for the valuation further clinico-paraclinical data were taken into consideration: proteinuria, nephrotic syndrome, arterial hypertension and the combination of nephrotic syndrome and arterial hypertension. At the beginning of the treatment 293 of 400 patients (73.2%) had a normal renal function, 107 of 400 patients (26.8%) were initially renal-insufficient. 16 of 400 patients (4.0%) had additionally a pyelonephritis and 12 of 400 patients (3.0%) had a lupus nephritis. 27 of 400 patients (6.9%) developed a terminal renal insufficiency after an average duration of observation of 40.5 months. Complications caused by therapy were registered in 104 of 400 patients (26.0%). Recommendations for the present therapy of chronic glomerulonephritis are formulated.

[Haptoglobin types and chronic glomerulonephritis]. / Haptoglobintyp und chronische Glomerulonephritis.

In 88 patients a chronic glomerulonephritis was diagnosed by histology and immunohistology. The haptoglobin type was also analyzed. The distribution of the haptoglobin types in the sample did not differ from the haptoglobin distribution in the population of Berlin. The distributions of histologic and immunohistologic forms of glomerulonephritis were related to the haptoglobin distribution in our patients. The distribution of histological subgroups does not depend on the distribution of the haptoglobins. In evaluation of the immunohistological subgroups we found that immunocomplexnephritis is less frequent in the group Hp 1--1 than in the groups 2--1 and 2--2. We suppose that persons with Hp 2--1 and Hp 2--2 are prone to increased antibody formation. Hp 1--1 is presumably a blocking antibody.