Biomedical subjects

Ettore Sabadini

Publications and source records attributed to Ettore Sabadini.

5 recordsLinked to original sources

PubMed2006

Renal involvement in Churg-Strauss syndrome.

BACKGROUND: Churg-Strauss syndrome (CSS) is a rare disorder characterized by asthma, eosinophilia, and systemic vasculitis. Renal involvement is not regarded as a prominent feature, and its prevalence and severity vary widely in published reports that usually refer to small series of selected patients. METHODS: We examined the prevalence, clinicopathologic features, and prognosis of renal disease in 116 patients with CSS. RESULTS: There were 48 men and 68 women with a mean age of 51.9 years (range, 18 to 86 years). Signs of renal abnormalities were present in 31 patients (26.7%). Rapidly progressive renal insufficiency was documented in 16 patients (13.8%); urinary abnormalities, 14 patients (12.1%); and chronic renal impairment, 1 patient. There were 3 additional cases of obstructive uropathy. Sixteen patients underwent renal biopsy, which showed necrotizing crescentic glomerulonephritis in 11 patients. Other diagnoses were eosinophilic interstitial nephritis, mesangial glomerulonephritis, and focal sclerosis. Antineutrophil cytoplasmic antibody (ANCA) was positive in 21 of 28 patients (75.0%) with nephropathy versus 19 of 74 patients without (25.7%; P < 0.001). In particular, all patients with necrotizing crescentic glomerulonephritis were ANCA positive. After a median follow-up of 4.5 years, 10 patients died (5 patients with nephropathy) and 7 patients developed mild chronic renal insufficiency. Five-year mortality rates were 11.7% (95% confidence interval, 3.9 to 33.3) in patients with nephropathy and 2.7% (95% confidence interval, 0.7 to 10.7) in those without (P = 0.10). CONCLUSION: Renal abnormalities are present in about one quarter of patients with CSS. The prevailing picture is ANCA-associated necrotizing crescentic glomerulonephritis; however, other forms of nephropathy also may occur. Outcome and long-term follow-up usually are good.

Adolescent↗

PubMed2005

Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome: a comparison of different assays.

BACKGROUND: The role of anti-glomerular basement membrane (GBM) antibodies in the pathogenesis of Goodpasture syndrome (GPS) is firmly established. Untreated, the disease may follow a fulminating course. Early identification of patients has important implications in terms of management and prognosis. Therefore, a diagnostic test for the determination of circulating anti-GBM antibodies, of very high sensitivity and specificity, is necessary. A number of assays, using different antigenic substrates, are available, but studies comparing the 'performances' of the different tests are scarce. METHODS: The aim of our work was to evaluate the sensitivity and specificity of four immunoassay-based anti-GBM antibodies kits. Thirty-four serum samples from 19 GPS patients, 41 pathological and 28 normal controls were studied retrospectively (the follow-up samples were not included in the analysis of performance data). Cut-off limits were derived from receiver operating characteristics curve analysis. RESULTS: All the assays showed a comparable good sensitivity (between 94.7 and 100.0%), whereas specificity varied considerably (from 90.9 to 100.0%). The better performance in terms of sensitivity/specificity was achieved by a fluorescence immunoassay which utilizes a recombinant antigen. CONCLUSION: All the assays have a good performance, with high sensitivity; however, the specificity may vary considerably.

Anti-Glomerular Basement Membrane Disease↗

PubMed2005

Value of a new automated fluorescence immunoassay (EliA) for PR3 and MPO-ANCA in monitoring disease activity in ANCA-associated systemic vasculitis.

The value of anti-neutrophil cytoplasmic antibody (ANCA) detection for monitoring disease activity in ANCA-associated systemic vasculitis (AASV) remains controversial. The aim of our work was to rate the performance of a new automated fluorescence PR3 and MPO-ANCA immunoassay (EliA) for monitoring disease activity in AASV. We evaluated 100 serum samples from 71 AASV patients (with Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome) as well as sera from 58 pathological and 35 normal controls. In addition to PR3 and MPO-ANCA EliA, we performed indirect immunofluorescence and "homemade" PR3 and MPO-ANCA ELISA tests. In AASV patients, ANCA levels were correlated with disease activity, according to the Birmingham Vasculitis Activity Score (BVAS). We derived cutoff limits from receiver operating characteristic (ROC) curve analysis comparing AASV with pathological controls. Our results showed that EliA and ELISA had comparable sensitivity (76%) and specificity (95%). The analysis of active versus inactive status and correlation with ANCA levels showed a clear difference between BVAS Group I (score < or = 4) and BVAS Group II (scores > 4) (AUC = 0.86 vs. 0.72; relative risk [RR] = 2.4; P < 0.0001) for PR3-ANCA, but not for MPO-ANCA (AUC = 0.94 vs. 0.87; RR = 1.48; P = 0.46). Serial serum samples from 16 patients were examined in detail. For the majority of patients, for both PR3 and MPO-ANCA, change in titer was strongly associated with change in BVAS score. Our data showed a good correlation between ANCA titer (especially for PR3) and AASV disease activity. We recommend that ANCA titer be used to monitor AASV disease activity with the caveat that a few exceptions, in particular with MPO-ANCA, are possible.

Antibodies, Antineutrophil Cytoplasmic↗

PubMedDate not supplied

The use of laboratory tests in diagnosis and monitoring of systemic lupus erythematosus.

Clinical immunology laboratories play an essential role in diagnosis and monitoring of systemic lupus erythematosus (SLE). To obtain the best results in terms of diagnostic performance and clinical usefulness, the following recommendations should be fulfilled: Indirect immunofluorescence on Hep-2 cells remains the method of choice for the detection of anti-nuclear antibodies (ANA). The sensitivity of ANA test for SLE is very high (almost 100%) but its specificity low since ANA can be present in a number of different clinical conditions and even in normal controls. Anti-dsDNA antibodies are highly specific for SLE and present in a high proportion of SLE patients (40-80%). The method of choice for anti-ds DNA is the Farr assay; however, the necessity of using radioactive material decreases its applicability. As an alternative, immunofluorescence on Crithidia Luciliae can be used in the diagnostic phase for its high specificity. It is not advisable to use ELISA, in the diagnostic phase, due to its low specificity. The quantitative determination of anti-dsDNA is useful for monitoring patients, in particular in the presence of nephritis. For monitoring, a quantitative method should be used (Farr assay or ELISA). The detection of antibodies to extractable nuclear antigens (ENA) and to phospholipids (Lupus anticoagulant and anti-cardiolipin antibodies with a beta2 glycoprotein I-dependent method) are useful to identify subgroups of patients at risk for some clinical manifestations (i.e. anti-phospholipid syndrome). New assays (anti-C1q and anti-nucleosome antibodies) have been recently proposed for diagnosis (anti-nucleosome) and monitoring SLE patients (anti-C1q and anti-nucleosome antibodies), with promising results. Among biological parameters, urinary levels of monocyte chemoattranct protein 1 (MCP1) seem to be the most useful to monitor nephritis activity in lupus patients.

Clinical Laboratory Techniques↗

PubMedDate not supplied

Azathioprine hypersensitivity: report of two cases and review of the literature.

Azathioprine (AZA) is a widely-used drug in the treatment of different diseases such as vasculitis, systemic lupus erythematosus, rheumatoid arthritis, inflammatory bowel diseases and in renal transplantation. Side effects of AZA can be classified as toxic, mainly dose related (myelosuppression and hepatotoxicity) and idiosyncratic, mainly dose independent. While the toxic effects are common and well documented, the hypersensitivity reactions are rare and it is not often easy to distinguish them from systemic sepsis or disease recurrence. We report two cases of AZA hypersensitivity occurring in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis each mimicking a vasculitis relapse or a septic complication of immunosuppression, as well as a review of the literature.

Adult↗