Glycosylation of proteinase 3 (PR3) is not required for its reactivity with antineutrophil cytoplasmic antibodies (ANCA) in Wegener's granulomatosis.

OBJECTIVE: The glycosylation status of autoantigens appears to be crucial for the pathogenesis of some autoimmune diseases, since carbohydrates play a crucial role in the distinction of self from non-self. Proteinase 3 (PR3), the main target antigen for anti-neutrophil cytoplasmic antibodies (ANCA) in patients with Wegener's granulomatosis (WG), contains two Asn-linked glycosylation sites. The present study explores the influence of the glycosylation status of PR3 on the PR3 recognition by ANCA in a well characterized population of patients with WG. METHODS: Forty-four patients with WG (459 serum samples) who participated in a multicenter randomized trial, were tested by capture ELISA for ANCA against PR3 and deglycosylated recombinant variants of PR3. RESULTS: The patients were followed for a median of 27 months, and the median number of serum samples per patient was 10. At baseline, the correlation between the levels of ANCA against PR3 and against all the deglycosylated recombinant variants of PR3 were greater than 0.94 (?<0.001 for all the comparisons). Longitudinal analyses comparing the levels of ANCA against PR3 versus all the deglycosylated recombinant variants of PR3, using linear mixed models, showed no significant statistical differences (rho >or=0.90 in all cases). CONCLUSION: The glycosylation status of PR3 has no impact on its recognition by ANCA in WG.

Agreement of anti-neutrophil cytoplasmic antibody measurements obtained from serum and plasma.

Serum and plasma are used interchangeably to measure anti-neutrophil cytoplasmic antibodies (ANCA), even though the release of ANCA target antigens during the preparation of serum could affect ANCA assays and cause discrepancies between the results obtained from serum and plasma. To what extent ANCA test results obtained from serum agree and correlate with results from plasma remains unknown. Therefore, a comprehensive comparison was performed using serum and plasma samples which were collected in 175 patients with active Wegener's granulomatosis at enrollment of a recent randomized trial. These paired serum and plasma samples were subjected to parallel ANCA testing by standard indirect immunofluorescence on ethanol-fixed neutrophils, a direct enzyme-linked immunoassay (ELISA) for proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, and two different capture ELISAs for PR3-ANCA. The concordance of categorical serum and plasma ANCA results was assessed using kappa-coefficients. These were > 0.8 for all assays, indicating a very good concordance between positive and negative serum and plasma results. Spearman's correlation coefficients for serum and plasma PR3-ANCA values obtained by direct ELISA and both capture ELISAs were > or = 0.95 (P < 0.0001). Our study shows that serum and plasma samples can be used interchangeably for measuring ANCA.

Isolated tricuspid valve disease in antiphospholipid syndrome.

Cardiac valve involvement in antiphospholipid syndrome (APS) has been consistently shown. However, isolated tricuspid valve disease within this syndrome has only been recently reported. We report a patient with primary antiphospholipid syndrome who had isolated tricuspid organic valve disease, pulmonary hypertension and pulmonary thromboembolism. We suggest that the APS should be listed as one of the aetiologies of organic isolated tricuspid valve disease in the adult population.

[Use of transjugular intrahepatic portosystemic shunt (TIPS) in an 84 year-old patient during acute variceal bleeding]. / Utilizacion del shunt portosistemico intrahepatico transyugular (TIPS) en un paciente 84 años durante un episodio de hemorragia aguda por varices esofagicas.

The transjugular intrahepatic protosystemic shunt (TIPS) is a nonsurgical method with low mortality which does not interfere with subsequent liver transplantation. We describe the case of an old male patient with chronic hepatopathy who was admitted because of an episode of variceal bleeding. Due to the failure of the medical treatment and sclerotherapy, coronarian stomachic vein was embolized with a coil and a TIPS was performed during the acute episode of variceal bleeding. He evolved favorably and after a year of the procedure remains asymptomatic.

Rupture of mitral chorda tendinea following blunt chest trauma.

Acute mitral insufficiency, originated from rupture of mitral chordae tendineae secondary to nonpenetrating thoracic trauma, is an unusual condition. This diagnosis is difficult to establish because physical examination, electrocardiogram, and cardiac enzymes are neither sensitive nor specific. The diagnosis of rupture must be quickly established because this disorder may be fatal. This paper reports the case of a patient with acute mitral insufficiency secondary to a mitral valve chord rupture a week after a nonpenetrating thoracic trauma.

Case report: reactive hemophagocytic syndrome associated with disseminated strongyloidiasis.

The reactive hemophagocytic syndrome is a condition characterized by systemic proliferation of benign hemophagocytic histiocytes, fever, cytopenia, abnormal liver function, and frequently coagulopathy and hepatosplenomegaly. Its occurrence has been documented in association with viral, bacterial, fungal and parasitic infections; a wide spectrum of malignant neoplasms; some miscellaneous disorders; and phenytoin. Disseminated strongyloidiasis is reported in a patients with systemic lupus erythematosus treated with corticosteroids in whom a reactive hemophagocytic syndrome developed and who finally died. This reactive hemophagocytic syndrome is reported for the first time in strongyloidiasis and may not have been recognized in former patients.