The impact of race on hospitalization outcomes for goodpasture's syndrome in the United States: nationwide inpatient sample 2003-2014.

BACKGROUND: Goodpasture's syndrome is a rare and life-threatening autoimmune disease. While Goodpasture's syndrome is well described in Caucasian and Asian populations, its prevalence and outcomes among African American and Hispanic populations are unclear. We conducted this study to assess the impacts of race on hospital outcomes among patients with Goodpasture's syndrome. METHODS: The National Inpatient Sample database was used to identify hospitalized patients with a principal diagnosis of Goodpasture's syndrome from 2003 to 2014. Goodpasture's syndrome patients were grouped based on their race. The differences in-hospital supportive care for organ failure and outcomes between Caucasian, African American, and Hispanic Goodpasture's syndrome patients were assessed using logistic regression analysis. RESULTS: Nine hundred and sixty-four patients were hospitalized with a primary diagnosis of Goodpasture's syndrome. Of these, 786 were included in the analysis: 622 (79%) were Caucasian, 73 (9%) were African American, and 91 (12%) were Hispanic. Hispanics had significantly lower use of plasmapheresis. The use for mechanical ventilation, noninvasive ventilation support, and renal replacement therapy in African Americans and Hispanics were comparable to Caucasians. There was no significant difference in organ failure, sepsis, and in-hospital mortality between African Americans and Caucasians. In contrast, Hispanics had higher in-hospital mortality than Caucasians but similar risk of organ failure and sepsis. CONCLUSION: African American and Hispanic populations account for 9% and 12% of hospitalizations for Goodpasture's syndrome, respectively. While there is no significant difference in in-hospital mortality between African Americans and Caucasians, Hispanics with Goodpasture's syndrome carry a higher in-hospital mortality compared to Caucasians.

The association of HLA-DQB1, -DQA1 and -DPB1 alleles with anti- glomerular basement membrane (GBM) disease in Chinese patients.

BACKGROUND: Human leukocyte antigen (HLA) alleles are associated with many autoimmune diseases, including anti-glomerular basement membrane (GBM) disease. In our previous study, it was demonstrated that HLA-DRB1*1501 was strongly associated with anti-GBM disease in Chinese. However, the association of anti-GBM disease and other HLA class II genes, including HLA-DQB1, -DQA1,-DPB1 alleles, has rarely been investigated in Asian, especially Chinese patients. The present study further analyzed the association between anti-GBM disease and HLA-DQB1, -DQA1, and -DPB1 genes. Apart from this, we tried to locate the potential risk amino acid residues of anti-GBM disease. METHODS: This study included 44 Chinese patients with anti-GBM disease and 200 healthy controls. The clinical and pathological data of the patients were collected and analyzed. Typing of HLA-DQB1, -DQA1 and -DPB1 alleles were performed by bi-directional sequencing of exon 2 using the SeCoreTM Sequencing Kits. RESULTS: Compared with normal controls, the prevalence of HLA-DPB1*0401 was significantly lower in patients with anti-GBM disease (3/88 vs. 74/400, p = 4.4 × 10-4, pc=0.039). Comparing with normal controls, the combination of presence of DRB1*1501 and absence of DPB1*0401 was significantly prominent among anti-GBM patients (p=2.0 × 10-12, pc=1.7 × 10-10). CONCLUSIONS: HLA-DPB1*0401 might be a protective allele to anti-GBM disease in Chinese patients. The combined presence of DRB1*1501 and absence of DPB1*0401 might have an even higher risk to anti-GBM disease than HLA-DRB1*1501 alone.

FCGR2B gene polymorphism rather than FCGR2A, FCGR3A and FCGR3B is associated with anti-GBM disease in Chinese.

BACKGROUND: The Fcgamma receptors play important roles in anti-glomerular basement membrane antibody disease (anti-GBM disease) in animal models, and FCGR gene polymorphisms have been reported to be associated with numerous human autoimmune diseases. We aimed to clarify the genetic association of FCGR gene polymorphisms with anti-GBM disease in Chinese patients. METHODS: A total of 48 patients with anti-GBM disease and 225 geographically and ethnically matched healthy controls were involved. Genotyping of the previously identified polymorphisms FCGR2A131H/R (rs1801274), FCGR2B 232I/T (rs1050501) and FCGR3A176F/V (rs396991) were detected by the TaqMan genotyping assay and FCGR3B NA1/2 by the PCR-sequence specific primer (SSP). Allele type, genotype and haplotype of identified polymorphisms were analysed between patients and controls. RESULTS: Our results revealed that FCGR2A131H/R, FCGR3A176F/V and FCGR3B NA1/2 were not associated with anti-GBM disease. The frequency of the FCGR2B 232T allele (30.2% versus 15.6%, corrected P = 0.00028, 95% CI: 1.42-3.89) and genotypes of I232T (60.4% versus 31.1%, corrected P = 0.0004, 95% CI: 1.78-6.43) was significantly increased in patients compared with controls. CONCLUSION: The present study demonstrates the genetic association of polymorphism of FCGR2B (I232T) with susceptibility to anti-GBM disease in Chinese.

Incidence and outcome of antiglomerular basement membrane disease in Chinese.

BACKGROUND: Antiglomerular basement membrane (anti-GBM) disease is an uncommon disease, especially among Asian population. Many reports and studies on this condition in the Caucasian population are available, but little information exists on anti-GBM disease in Asians. To study the incidence and clinical characteristics of anti-GBM disease among Chinese patients, we reviewed our experience of anti-GBM disease in our hospital (Queen Mary Hospital, Hong Kong) from 1992 to 2003. METHODS: All patients who were admitted for acute renal impairment, which was caused by crescentic glomerulonephritis associated with linear immunoglobulin G (IgG) staining on immunofluorescence, were included in the analysis. Serum anti-GBM antibodies were detected by either enzyme-linked immunofluorescence or indirect immunofluorescence. Ten patients were treated for anti-GBM disease during this 11-year period, yielding an incidence of approximately 0.6 cases per million population per year. RESULTS: In this cohort, anti-GBM disease predominantly affected older patients (mean age: 58.6 +/- 21.7 years). Eight patients were aged between 60 and 80 years and there was a female preponderance (M:F = 2:8). The 1-year renal and patient survival was 15% (95% CI 0-40%) and 70% (95% CI 42-98%), respectively. Most patients presented with non-specific symptoms as well as impaired renal function. Detection of anti-GBM antibody provided a good screening test for the disease. Antiglomerular basement membrane antibodies were not detected in two patients. All but two patients received steroid, cyclophosphamide and intensive plasmapheresis therapy. Haemoptysis occurred in four patients (40%), and usually lagged behind the renal presentation and commencement of treatment. Six patients required long-term dialysis after the acute disease. Three patients died from the disease, two died from pulmonary complications and one died suddenly after a partial recovery of renal function. CONCLUSION: Antiglomerular basement membrane disease is uncommon among the Chinese population. It predominantly affects older patients, and prognosis is poor. Long-term preservation of renal function after the initial attack is unusual.