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Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study.

ABSTRACT: Perinuclear Anti Neutrophil Cytoplasmic Antibody (p-ANCA) is a serological marker of Microscopic Polyangiitis (MPA), a vasculitis associated with lung involvement potentially mimicking Idiopathic Pulmonary Fibrosis (IPF). In this study, we evaluated the role of p-ANCA in predicting clinical evolution and prognosis in a cohort of IPF patients. In this observational, retrospective, case-control study, we compared 18 patients with an IPF diagnosis and p-ANCA positivity with 36 patients with seronegative IPF, matched for age and sex. IPF patients with and without p-ANCA showed similar lung function decline during the follow-up, but IPF p-ANCA+ showed better survival. Half of IPF p-ANCA+ patients were classified as MPA for the development of renal involvement (55%) or skin signs (45%). The progression towards MPA was associated with high levels of Rheumatoid Factor (RF) at baseline. In conclusion, p-ANCA, mainly when associated with RF, could predict the evolution of Usual Interstitial Pneumonia (UIP) towards a definite vasculitis in patients, with a better prognosis compared with IPF. In this view, ANCA testing should be included in the diagnostic workup of UIP patients.

SUBMITTER: Libra A

PROVIDER: S-EPMC10253038 | biostudies-literature | 2023 May

REPOSITORIES: biostudies-literature

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Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study.

Libra Alessandro A Muscato Giuseppe G Ielo Giuseppe G Spicuzza Lucia L Palmucci Stefano S Fagone Evelina E Fruciano Mary M Gili Elisa E Sambataro Gianluca G Vancheri Carlo C

Diagnostics (Basel, Switzerland) 20230527 11

Perinuclear Anti Neutrophil Cytoplasmic Antibody (p-ANCA) is a serological marker of Microscopic Polyangiitis (MPA), a vasculitis associated with lung involvement potentially mimicking Idiopathic Pulmonary Fibrosis (IPF). In this study, we evaluated the role of p-ANCA in predicting clinical evolution and prognosis in a cohort of IPF patients. In this observational, retrospective, case-control study, we compared 18 patients with an IPF diagnosis and p-ANCA positivity with 36 patients with seroneg ...[more]

PMID: 37296734

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Project description:INTRODUCTION:Idiopathic pulmonary fibrosis(IPF) is chronic fibrosing interstitial pneumonia of unknown aetiology. IPF is diagnosed based on the exclusion of known causes such as connective tissue diseases(CTDs). However, some patients fail to meet defined CTD criteria regardless of an implication of immunological involvement and these cases were described in a variety of terms. The classification criteria of this clinical entity consist of a combination of clinical, serological and morphological findings and it is reported to be distinct from IPF. However, the significance of the sole presence of autoantibodies complicated with IPF is still unknown. Therefore, this systematic review aims to clarify the significance of autoantibodies complicated with IPF. METHODS AND ANALYSIS:IPF with any autoantibody associated with CTDs is eligible for the review. Primary outcomes are all-cause mortality and pulmonary-cause mortality, while secondary outcomes include a progression of the disease, a deterioration of health-related quality of life and the development of a defined CTD. Primary studies of any type except a case report are included. Two reviewers search four electronic databases such as Medline, EMBASE, Science Citation Index Expanded and Google Scholar from each inception through 1 February 2018 and extract data independently. A risk of bias in individual studies is assessed by the Quality in Prognostic Studies tool. Meta-analysis is sought to be conducted if three or more studies report an outcome for a specific autoantibody with the same statistics. If it is inappropriate to combine data due to substantial heterogeneity, the result is reported qualitatively. Subgroup and sensitivity analyses are considered to identify the source of heterogeneity. The Grades of Recommendation, Assessment, Development and Evaluation method is applied to evaluate the evidence level of the result. ETHICS AND DISSEMINATION:There is no concerning ethical issue. The result will be sought for publication. PROSPERO REGISTRATION NUMBER:CRD42017077336.

Project description:BackgroundDespite its heterogeneity, there is currently limited data in pleural infection phenotyping. Using pleural fluid characteristics, pleural infection can be classified into microbiological-positive pleural infection (MPPI) and microbiological-negative pleural infection (MNPI). This study aimed to evaluate the prognostic significance of microbiological positivity in pleural infection, and to evaluate the performance of RAPID (renal, age, purulence, infection source, dietary factor) score in these subgroups.MethodsConsecutive patients hospitalized for pleural infection over a 10-year period in two acute-care hospitals in Hong Kong were evaluated. According to the pleural fluid characteristics, they were classified into MPPI and MNPI, respectively. Survival was evaluated using multivariate Cox regression analysis. Performance of RAPID score to predict mortality at 3-month and 1-year was evaluated using C-statistics.ResultsIn total, 381 patients with pleural infection were included. They were classified into MPPI (n = 169) and MNPI (n = 212), respectively. The MPPI group had more elderly home residence and use of large-bore chest tube, and higher Charlson comorbidity index and RAPID score, compared to the MNPI group. Length-of-stay, the need of surgery and intensive care were similar between the two groups. MPPI was associated with significantly increased risk of mortality (adjusted hazard ratio [aHR] 1.46, 95% CI 1.08-1.98). Three-month mortality was significantly higher in MPPI compared to MNPI (24.9% vs. 10.4%, p < 0.001; adjusted odd ratio 2.05, 95% CI 1.11-3.80). The trend continued at 1, 3, 5 and 7 years. RAPID score predicted 3-month and 1-year mortality in both groups (C-statistics, MPPI 0.71, 0.75; MNPI 0.84, 0.81). In the MPPI group, presence of Staphylococcus aureus (aHR 2.26, 95% CI 1.43-3.57) and Gram-negative organisms other than Enterobacteriaceae (aHR 2.00, 95% CI 1.10-3.61) were associated with worse survival, while presence of Streptococcus anginosus group was associated better survival (aHR 0.50, 95% CI 0.32-0.78), when compared to their absence.ConclusionsPleural fluid microbiological positivity is independently associated with increased mortality in patients with pleural infections. This finding should complement the RAPID score in risk stratification and inform future research aimed at improving outcomes in this patient population.

Project description:IntroductionInvestigations of muscle dysfunction in patients with idiopathic pulmonary fibrosis (IPF) are limited to peripheral muscles. However, decreased thoracic muscle mass is known and deterioration of chest wall muscle strength is not clear.ObjectiveThe aims of the present study were to evaluate pectoralis muscle strength located on the chest wall and to investigate the relationship of spirometric measurements and respiratory muscle strength with pectoralis muscle strength.MethodsElderly patient with IPF (mean disease duration 7.47±7.04 years) and the age-and sex-matched healthy volunteers were recruited in this cross-sectional study. The pulmonary function test was performed by a portable spirometer for spirometric variables and a gas analyzer for diffusing capacity for carbon monoxide (DLCO). Maximal inspiratory (MIP) and expiratory pressure (MEP) were measured with mouth pressure device. Modified Medical Research Council Dyspnea Scale (MMRC) was used to determined dyspnea severity. The pectoralis muscle strength was assessed isometrically during shoulder joint horizontal adduction movement with a handheld dynamometer.ResultsA total of 17 patients with IPF (9 males, mean age 69.06±3.94 years) and 19 healthy controls (10 males, mean age 70.95 ±4.99 years) were included. Patients with IPF had lower pectoralis muscle strength than healthy controls (p<0.001). Significant relationships were found between pectoralis muscle strength and MIP (r=0.79, p<0.001), MEP (r=0.81, p<0.001), FEV1% (r=0.54, p=0.02), FVC% (r=0.68, p<0.003) and DLCO (r=0.61, p=0.009). With multiple linear regression analysis, pectoralis muscle strength was the only independent predictor of FVC% (adjusted R2=0.37, p<0.05).ConclusionIn patients with IPF, pectoralis muscle strength decreases and is associated with pulmonary function. In particular pectoralis muscle strength is likely to have an important impact on FVC%. Therefore, we consider that this test should be included routinely in chest diseases and rehabilitation clinics. The trial was registered U.S. National Library of Medicine clinical trial registry (https://clinicaltrials.gov, Trial ID: NCT04803617).

Project description:ObjectiveTo clarify clinical significance of the sole presence of autoantibodies for idiopathic pulmonary fibrosis (IPF) without any other symptoms or signs suggestive of autoimmune disease.DesignSystematic review and meta-analysis DATA SOURCES: Medline, EMBASE, Science Citation Index Expanded and Google Scholar were searched from 1 January 2002 through 12 February 2019.Eligibility criteria for selecting studiesPrimary studies addressing all-cause mortality and the development of a defined autoimmune disease for IPF with autoantibodies were included for the review.Data extraction and synthesisTwo reviewers extracted relevant data and assessed risk of bias independently. Meta-analysis was conducted using a random-effects model if three or more studies reported the same outcome for a certain autoantibody. The quality of evidence was assessed by the Grades of Recommendation, Assessment, Development and Evaluation system.ResultsOut of 4603 records retrieved nine studies were included in this review. All studies contained some risk of bias. Based on pooled data myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was significantly associated with microscopic polyangiitis incidence with risk ratio (RR) of 20.2 (95% CI: 7.22 to 56.4) and antinuclear antibody (ANA) was also significantly associated with the development of connective tissue diseases with RR of 7.11 (p=0.001) (10 cases in 157 patients with ANA) in one study. However, there was no significant association of autoantibodies with all-cause mortality aside from MPO-ANCA and proteinase 3-ANCA in one study each. MPO-ANCA was not demonstrated to be associated with all-cause mortality by meta-analysis. The quality of evidence was deemed as either low or very low.ConclusionsThe presence of autoantibodies such as MPO-ANCA and ANA was demonstrated to be associated with the development of some autoimmune diseases for patients with IPF although there was no difference of all-cause mortality. However, the results should be interpreted with caution due to low evidence level.Prospero registration numberCRD42017077336.

Dataset Information

Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study.

Publications

Clinical and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study.

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