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1.
Semin Arthritis Rheum ; 48(4): 714-719, 2019 Feb.
Article in English | MEDLINE | ID: mdl-29891418

ABSTRACT

OBJECTIVES: Granulomatosis with polyangiitis-a primary systemic vasculitis-most frequent manifestations are respiratory. Airway disease can present with stenosis, and although subglottic stenosis (SGS) is well described, narrowing distal to the glottis has been more recently the focus of reporting. Our objectives, therefore, are to describe the frequency, characteristics, and treatment of tracheobronchial stenoses (TBS) in granulomatosis with polyangiitis (GPA) at our institution, a national referral center for respiratory diseases. Also, to identify factors associated with TBS development in GPA. METHODS: We undertook a retrospective study of all identified TBS cases (n-29) in whom their demographics, clinical and paraclinical features, and treatment were analyzed with descriptive statistics. Comparisons between those who developed and did not develop TBS (n-79) were established with the χ2 test with Yates correction or two-tailed exact Fisher test for comparisons among groups with or without this complication. RESULTS: Females were predominantly affected by TBS (76%). Most patients had limited GPA (n-20, 69%). TBS appeared at a mean of 29 months after diagnosis of GPA. Main symptoms were dysphonia, stridor, and dyspnea. All TBS patients had tracheal involvement and 12 (41%) additional bronchial stenoses. Other accompanying manifestations by organ/system were rhinosinusal (n-26, 87%), musculoskeletal (n-16, 55%), ocular (n-13, 45%), pulmonary (n-12, 41%), renal (n-8, 27%), mucocutaneous (n-5, 17%), neurological (n-4, 13%). At TBS diagnosis, 17 patients were PR3-ANCA and/or C-ANCA positive, while 9 were MPO-ANCA and/or P-ANCA positive; results on the remaining were either negative or ANCA were not performed at that time. Seventeen patients had simultaneous medical (8 with glucocorticoid therapy, 9 with immunosuppressants) and surgical therapy, while the rest only the latter at the time of TBS diagnosis. Relapses were frequent (one in 18 patients; two in 11, three in 9 and 2 individuals had ≥4 relapses). Factors positively associated with TBS were the presence of general and musculoskeletal symptoms and rhinosinusal disease, while those negatively associated were prednisone oral daily dose >10mgqd and azathioprine intake. The extent of disease, either generalized or limited was not associated with TBS development, nor were ANCA levels. CONCLUSIONS: TBS are serious complications of GPA which can arise at any stage of the disease. Timely diagnosis plus optimal treatment and follow-up remain unmet needs.


Subject(s)
Granulomatosis with Polyangiitis/complications , Tracheal Stenosis/complications , Adult , Female , Glucocorticoids/therapeutic use , Granulomatosis with Polyangiitis/drug therapy , Humans , Immunosuppressive Agents/therapeutic use , Male , Middle Aged , Recurrence , Retrospective Studies , Risk Factors , Tracheal Stenosis/drug therapy
3.
Ther Clin Risk Manag ; 13: 9-14, 2017.
Article in English | MEDLINE | ID: mdl-28031716

ABSTRACT

BACKGROUND: The term nodular is not included in the Sydney classification and there is no widely accepted histopathological definition. It has been proposed that the presence of antral nodularity could predict Helicobacter pylori (H. pylori) infection. The aim of this study was to determine the diagnostic accuracy of nodular gastritis (NG) for H. pylori infection after a rigorous standardization process, and to describe the associated histopathological characteristics. MATERIALS AND METHODS: Endoscopic images of patients submitted to endoscopy with biopsy sampling were included. Endoscopic images were distributed among six endoscopists. The analysis was performed sequentially in three rounds: the first round assessed the interobserver variability, the second evaluated the intraobserver variability, and the third calculated the interobserver variability after training. A correlation analysis between endoscopic and histopathological findings was performed. RESULTS: A total of 917 studies were included. In the first analysis of interobserver variability, a poor kappa value (0.078) was obtained. The second evaluation yielded good intraobserver variability, with kappa values of 0.62-0.86. The evaluation of interobserver variability after training revealed an improvement in the kappa value of 0.42. A correlation was found between endoscopic images and histopathological reports. CONCLUSION: There was a strong correlation between NG and H. pylori, but only after rigorous evaluation. The use of the term NG requires extensive standardization before it can be used clinically.

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