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A 68-year-old male with macroscopic hematuria and constitutional symptoms as fever and weight loss. There was nothing interesting in the anamnesis or in the physical exploration. The laboratory test had an elevation of creatinine of 4 mg/dL and ten days before it had been 1.4 mg/dL. In the urine analysis: proteinuria of 1.5 G/24 h, and hematuria. On the second day we made a renal biopsy where we could seen segmental glomerular necrosis and crescent fromation in 80% of the glomeruli. In the immune study c-ANCA anti-PR3 was positive. In the complementary studies we didn't find other organs affected. With the diagnosis of pauci-immune glomerulonephritis limited to the kidney we began treatment with corticosteroids and cyclophosphamide. As the renal function was severely affected the patient needed one dialysis session. We began with 1 g intravenous methylprednisolone daily for 3 days followed by oral prednisone 60 mg daily tapering to 10 mg daily by 3 months. This was combined with 150 mg oral cyclophosphamide daily. Seven plasma exchanges were performed. At the beginning of treatment creatinine was 7 mg/dL, it was decreasing rapidly and three week after cretinine was 3 mg/Dl and he was asymptomatic. One year after treatment, creatinine is 1.4 mg/dL and the urine analysis is normal, C-ANCA are negative.

Rapidly progressive glomerulonephritides (RPGN) are forms of necrotizing glomerulonephritis associated with anti-glomerular basement membrane (anti-GBM) and anti-neutrophil cytoplasmic antibodies (ANCA) against the antigens proteinase-3 (anti-PR3) and myeloperoxidase (anti-MPO). RPGN have a course of rapid progression to renal failure. We compared the results from the semiquantitative ELISAs for anti-GMB antibodies, PR3-ANCA and MPO-ANCA and the indirect immunofluorescence technique (IIF) against a new rapid assay (30 minutes) for the same antibodies in patients with clinically suspected RPGN. The semiquantitative ELISAs for anti-GBM antibodies and PR3-ANCA and MPO-ANCA have a proven diagnostic significance in patients with RPGN I and III. There were no significant differences between the ANCA-GBM screening test and the results from the semiquantitative ELISAs (p > 0.05). We did not find significant differences between the results for PR3-ANCA and MPO-ANCA from the ANCA-GBM screening test with C-ANCA and P-ANCA IIF values (p > 0.05). We also corroborated that the ANCA-GBM screening test is a diagnostic tool for RPGN I and III as useful as the semiquantitative ELISAs and the IFF technique. The ANCA-GBM ELISA screening test is a tool as useful as the semiquantitative ELISA against anti-GBM antibodies for diagnosis of RPGN I. The comparison of the screening ELISA with the IIF technique and the semiquantitative ELISAs against PR3-ANCA and MPO-ANCA showed similar utility for diagnosis of RPGN III. The advantages of the new screening assay are that three antibodies are tested at the same time, yielding results in only 30 minutes.

Purpose: To determine whether anti neutrophil cytoplasmic antibodies (ANCA): anti myeloperoxidase (anti MPO) and anti proteinase 3 (anti PR3), are present in patients with sickle cell anaemia (SCA) during painful crisis., Patients and Methods: 55 patients with SCA were included in this study, 35 of them had painful crisis and the rest were in steady state. We determined levels of anti MPO and anti PR3 by two enzyme immunoassays commercially available (Progen Biotechnik GMBH)., Results: A significant increase of anti MPO in the group of patients with painful crisis was found in comparison with steady state patients and controls., Conclusions: Our results suggest the possibility that anti MPO interacting with MPO on primed neutrophils lead to neutrophil activation, enhanced neutrophil adhesion and amplification of endothelial cell damage.

Background: Analysis of usefulness of antineutrophil cytoplasmic antibodies (AN-CA) as a marker of clinical activity in small vessel vasculitis., Patients and Methods: 33 patients, 10 patients with Wegener's granulomatosis (WG) and 23 with microscopic polyangiitis (MPA) and rapidly progressive glomerulonephritis type III (RPGN III). The clinic and serologic follow-up was accomplished every 3 months during an average of 19 (SD, 24) months (range 3-52 months. The serologic follow-up included the determination of ANCA by indirect immunofluorescence (IFI) and ELISA, as well as the serum level of C reactive protein (RCP)., Results: At the time of diagnosis all patients were ANCA positive by IFI and ELISA. The seroconversion of ANCA from positive to negative was produced in 30/33 patients (90%). Twenty-six out of these 30 patients (87%) achieved the seroconversion within the first 6 months. During the follow-up 4 patients had a major relapse, all with positive ANCA. In 2 patients, one of each group, seroconversion from negative to positive was not associated with clinical relapse of vasculitis., Conclusion: ANCA should be used in conjunction with other indices of disease activity in patients with small vessel vasculitis.

Mast cells play a central role in allergic diseases, following activation by means of immunologic mechanisms. Mast cell degranulation releases preformed and newly generated mediators and potential indicators of mast cell activation. Tryptase is the most useful marker of selective mast cell activation with regard to specific pathological states in which it can be applied with positive results, especially allergic diseases.

Background: The prevalence and diagnostic usefulness of antineutrophil cytoplasmic antibodies (ANCA) were analyzed in a series of patients with different types of vasculitis, connective tissue diseases, nephropathies and lung diseases., Methods: Six hundred seventy-six consecutive patients with systemic vasculitis, connective tissue diseases, nephropathies and different lung disturbances were included in the study. The ANCA were determined by indirect immunofluorescence (IIF) on neutrophils fixed in ethanol. For the detection of antimyeloperoxidase (MPO) antibodies (Ab) a new technique was developed consisting in the performance of IIF on neutrophils with a deficiency in MPO. The serologic samples were also analyzed by enzyme-linked immunosorbent assays with MPO and purified proteinase 3 (PR3)., Results: ANCA were demonstrated in 95 patients. A cytoplasmic pattern (c-ANCA) was detected in 25 cases (26%) and in 70 (74%) a perinuclear pattern (p-ANCA) was observed. Twenty-one of the 25 cases (84%) of c-ANCA corresponded to anti-PR3 Ab and 61 of the 70 (87%) of p-ANCA corresponded to anti-MPO Ab. The anti-PR3 Ab identified Wegener's granulomatosis, with a sensitivity and specificity for the generalized and clinically active forms being 65 and 88%, respectively. The anti-MPO Ab were principally detected in patients with rapidly progressive glomerulonephritis (RPGN) and hemorrhagic alveolar capillaritis. The sensitivity and specificity of the anti-MPO Ab for the RPGN and alveolar capillaritis were 75% and 98%., Conclusions: Two principal subtypes of ANCA may be detected, which also recognize two fundamental clinicopathologic entities. The high diagnostic sensitivity and specificity of these antibodies for each of the processes to which they are associated can justify the adoption of therapeutic measures in determined cases.