1,721,053 research outputs found
Mesalazine-induced Churg-Strauss syndrome in a patient with Crohn's disease and sclerosing cholangitis
No full textComparison of PR3-ANCA specific assay performance for the diagnosis of granulomatosis with polyangiitis (Wegener's)
No full textBackground: PR3-ANCA, the serological marker of granulomatosis with polyangiitis (GPA), is usually detected by immunometric assays, with purified PR3 directly coated onto the solid-phase. Novel methods for PR3-ANCA detection have been developed to improve the performance of traditional PR3-ANCA specific assays, but little is known about their diagnostic performance in real-life clinical settings. This study aimed to compare the performance of nine different commercial PR3-ANCA specific assays, including traditional and newer ones, for the diagnosis of GPA. Methods: The evaluated assays for PR3-ANCA detection were representative of the first, second, and third generation tests (direct, capture and anchor assays, respectively). A third-generation assay employing both human and recombinant PR3 was also evaluated. The study population consisted of 55 GPA patients, 175 disease controls (representing most diseases in differential diagnosis with primary small-vessel vasculitis) including 52 with microscopic polyangiitis, and 20 healthy subjects. We performed the primary evaluation of test sensitivity using cut-off points which provided adequate and identical specificity for each test. Results: Although specificity and area under the ROC curve did not differ significantly between the different assays, substantial differences in sensitivity at 98%-specificity were found in some instances (p<0.001). Compared to first generation direct PR3-ANCA specific assays, some of the second and third generation tests increased the positive predictive value (PPV) for GPA diagnosis. Conclusions: Some of the newer PR3-ANCA specific assays have better PPV than traditional ones
Otorhinolaryngological manifestationsin granulomatosis with polyangiitis (Wegener's)
No full textGranulomatosis with polyangiitis (Wegener's, GPA) is an uncommon disease of unknown etiology classically involves the ELK triad of the ear, nose, throat (E), lungs (L) and kidneys (K) with necrotizing granulomatous inflammation and vasculitis. Most of the initial symptoms begin in the head and neck region with a wide spectrum of involvement of any site ranging from the nasal septum, paranasal sinuses, oral mucosa, larynx and even the external, middle and internal ear. Diagnosis may be delayed because the onset is heterogeneous and sometimes limited to one organ. The pathologic findings of a characteristic inflammatory reaction pattern, and the serum findings of elevated antineutrophil cytoplasmic antibodies can help to establish the diagnosis. The differentiation from other conditions that mimic GPA such as lymphoma and infections is of critical importance to initiate appropriate treatment. Treatment of the underlying disease is medical with the use of immunosuppressive agents and will not be reviewed here. This review focuses on the otorhinolaryngologic manifestation and complication of GPA as well as their surgical management and specifies the role of the otorhinolaryngologist as an integral member of the multidisciplinary care team for patients with GPA
Meningeal involvement in apparently ANCA-negative Wegener's granulomatosis: a role for PR3 capture-ELISA?
No full text
Comparison of PR3-specific ANCA assays performance for diagnosis of granulomatosis with polyangiitis (GPA)
No full textChurg–Strauss syndrome
No full textA 51-year-old Caucasian man was hospitalized because
of myalgia and fever. He had been suffering from
chronic rhinitis since the age of 18 years and from asthma
since the age of 45 years. Three months before
hospitalization, he had received an influenza vaccine. On
admission, he also complained of fatigue and paresthesias
involving the lower limbs, and reported the recent onset
of palpable purpura at both legs (Figure 1a). Laboratory
tests are summarized in Table 1. The patient’s HLA-DRB1
genotype was positive for *04–*07 alleles, both belonging
to the HLA-DRB4 gene. Chest computed tomography (CT)
scan was normal, whereas head CT showed diffuse sinusitis
(Figures 1c and d). Electroneurography disclosed
sensorimotor polyneuropathy with signs of axonal damage
affecting the right peroneal and left sural nerves. A biopsy
of the purpuric lesions was performed, and histology
showed leukocytoclastic vasculitis (Figure 1b). As an
antineutrophil cytoplasmic antibody (ANCA)-associated
vasculitis was suspected and urinary abnormalities
persisted, renal biopsy was performed. On light microscopy
(Figure 2), the biopsy specimen included 24 glomeruli,
3 of which were obsolescent. Segmental necrosis was
found in 30% of the glomeruli, whereas four showed
extracapillary proliferation. The tubulointerstitium,
arterioles, and venules were normal, with no eosinophilic
infiltration. Immunofluorescence showed no immune
deposits. Churg–Strauss syndrome (CSS) was diagnosed on
the basis of histological findings showing vasculitis and the
presence of asthma, eosinophilia, sinusitis, and
polyneuropathy. Prednisone therapy (initial dose 1mg/kg/
day) induced rapid symptom remission, normalization
of the eosinophil count, and urinary abnormalities.
Prednisone was stopped 9 months later but was resumed
soon after withdrawal because of relapsing asthm
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