A Clinical Analysis Of Patients With Anti-Glomerular Basement Membrane Antibody Related Diseases

ObjectiveTo investigate the clinical and pathological characteristics of anti-glomerular basement membrane (GBM) antibody associated diseases with different clinical patterns, and to deepen the comprehension of the diseases and raise the early diagnosis and cure rate.MethodsThe clinical and pathological data of 22 patients with anti-GBM antibody associated diseases diagnosed in renal institute of Shandong Provincial Hospital in the last five years, were retrospectively analyzed.Results1. The severities of diseases of 22 patients were different. 21 patients were manifested as acute nephritis syndrome with rapidly progressive renal failure and anima, 1 patient with normal renal functions, 8 cases with pneumorrhagia.2. Of the 22 patients, 11 ones had positive anti-GBM antibody only, 7 ones had both anti-GBM antibody and antineutrophil cytoplasmic antibody (ANCA) positive, and 4 ones had both anti-GBM antibody and anti-nuclear antibody (ANA) positive. In the third group, one patient had anti-double-strand-DNA (ds-DNA) antibody positive, meanwhile, another with both anti-ds-DNA antibody and anti-Smith (Sm) antibody positive.In the group of both anti-GBM antibody and ANCA positive , the average age was older and the average level of anti-GBM antibody was lower than the other two groups, but there was no statistical significance. There were also no significant differences at the efficiency of treatment among the three groups.3. 11 of the 13 patients with renal biopsy had crescentic glomerulonephritis, 6 of whom had more than 85% of glomerli affected by crescents. As to the remaining 2 patients, one presented with chronic sclerosing glomerulonephritis, and the other was with mild mesangial proliferative glomerulonephritis.With immunofluorescence examinations, none of the 13 patients had typical IgG and C3 linear deposition along glomerular capillary walls. 8 patients had immunoglobulins and/or complements granula deposition along glomerular capillary walls and in mesangium, and 3 ones were with negative immunoinfluorescence.4. There were no significant differences at the level of anti-GBM antibody at the time of definite diagnosis, among the complete remission, improved and ineffective groups by immunosuppressive treatment.Conclusions1. The severities of manifestions of the patients of anti-GBM antibody associated diseases are at different extent. A few patients are with normal renal functions or mild kidney injury.2. A few patients have positive ANA, anti-ds-DNA antibody, or anti-Sm antibody, implying that some patients of lupus nephritis may have positive anti-GBM antibody.3. The renal immunofluorescence patterns are not all typical IgG and C3 linear deposition along glomerular capillary walls, and some patients are with granula deposition.4. There may be no correlation between the prognosis and the level of anti-GBM antibody in the patients with anti-GBM antibody associated diseases. In order to improve prognosis, it is important to make early diagnosis and timely intensive immunosuppressive treatment.