Clin Exp Rheumatol
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Multicenter Study
Intra- and inter-rater reliability of endonasal activity estimation in granulomatosis with polyangiitis (Wegener´s).
Granulomatosis with polyangiitis (GPA) frequently starts with an affection of the nasal and paranasal mucosa. Localised GPA of the nasal mucosa or persistent disease activity ('grumbling disease') is often encountered even under immunosuppressive therapy. Necessity for reconstructive surgery is common and careful scheduling to prevent failure and minimise revision rates is crucial. Therefore, reliable estimation of GPA activity in the upper airways using a score is mandatory for diagnosis, follow-up and scheduling reconstructive surgery. ⋯ Intra-rater reliability is high in decision in dichotomy and even in grading activity. There is no difference for experienced or inexperienced physicians. Inter-rater reliability is high in dichotomy, but low for activity grading. Thus, the ENTAS provides a reliable instrument for assessing, documenting and following GPA-related disease activity in the upper respiratory tract. The relationship of activity and following damage needs to be investigated in further studies.
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Comparative Study
Comparative analysis of different commercial ELISA systems for the detection of anti-neutrophil cytoplasm antibodies in ANCA-associated vasculitides.
To assess the diagnostic performance of 11 commercial PR3- and MPO-ANCA ELISA systems (direct, capture and high sensitive [hs] ELISA). ⋯ Second (capture) and third (high sensitivity) generation PR3-ANCA ELISA kits are superior to conventional ELISAs. Direct and capture MPO-ANCA ELISAs showed a good overall performance in all kits. Most of the kits have not been standardised to allow their results to be compared.
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The Patient Acceptable Symptom State (PASS) is the value beyond which patients consider themselves well. Our aim was to determine the PASS estimate for patients with AS by assessing pain, disease activity and functional impairment and evaluate whether the PASS is stable over time. ⋯ A high percentage of Moroccan patients with AS (55%) reported being in PASS. PASS threshold for pain was unexpectedly high, possibly suggesting a high level of patients' tolerance to pain.
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Plasma exchange (PE) has been shown to improve renal outcome in anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and severe renal failure; however the effect of PE in AAV with moderate renal impairment is controversial. ⋯ This study suggests the use of PE in addition to standard induction treatment with cyclophosphamide and glucocorticoids to patients with renal PR3-AAV and an estimated-GFR <60 ml/min/1.73m2.
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Alveolar haemorrhage (AH) can be a mild or life-threatening manifestation of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), but its prognostic impact and specific characteristics remain controversial. Our objective was to determine the prognostic value of AH in this context. ⋯ As previously demonstrated by the Five-Factor Score, AH alone is not predictive of poor prognosis, unlike kidney involvement, which dictates a poor outcome.