scholarly journals Mucins MUC5B and MUC5AC in Distal Airways and Honeycomb Spaces: Comparison among Idiopathic Pulmonary Fibrosis/Usual Interstitial Pneumonia, Fibrotic Nonspecific Interstitial Pneumonitis, and Control Lungs

2016 ◽  
Vol 193 (4) ◽  
pp. 462-464 ◽  
Author(s):  
Caterina Conti ◽  
Angeles Montero-Fernandez ◽  
Elaine Borg ◽  
Tina Osadolor ◽  
Patrizia Viola ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Interstitial Pneumonitis ◽  
Usual Interstitial Pneumonia ◽  
And Control

AB0528 CHARACTERIZATION OF ANTI-MPO POSITIVE INTERSTITIAL LUNG DISEASE. CLINICAL-SEROLOGIC AND RADIOLOGIC FEATURES AND SURVIVAL

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1561-1562
Author(s):  
G. Cassone ◽  
G. Dei ◽  
G. Sambataro ◽  
A. Manfredi ◽  
S. Cerri ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Pulmonary Involvement ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Anca Associated Vasculitis ◽  
Radiologic Features

Background:Prevalence of anti-neutrophil cytoplasmic antibody (ANCA) in patient with idiopathic pulmonary fibrosis (IPF) ranges from 1 to 35%, mainly anti-MPO. The presence of ANCA positivity seems to be a poorer prognostic factor in patient with IPF, and some of these patients will develop clinical vasculitis (7-23%).Unfortunately, the majority of the available studies on this topic are retrospective and the real natural history of the disease remains poorly understood.Objectives:Aim of the study was to investigate the clinical, serological and radiologic features of patients with interstitial lung disease (ILD) and positivity for anti-MPO, and to evaluate the survival of this population compared with IPF patients.Methods:We retrospectively analysed 30 patients with ILD and anti-MPO antibodies, without diagnosis of vasculitis, from 3 different rheumatology-pulmonology Italian Center.For each patient, clinical, radiologic and serological data were evaluated. Treatments were also collected, both immunosuppressants or antifibrotic agents.Finally, survival of ILD-MPO patients and of 90 unselected idiopathic pulmonary fibrosis (IPF) patients was compared.Results:Thirty patients were enrolled in the study (see table for the characteristics of the patients).Fibrosing pneumonia was described in 73.3% of patients (usual interstitial pneumonia [UIP] in 19 patients), and 10 patients (33.3%) received antifibrotic drugs, all with UIP pattern. Of interest, 7 patients were treated with immunosuppressants (azathioprine, cyclophosphamide, mycophenolate mofetil), independently by the ILD pattern and 21 (70%) low dosage of steroids.After a median period of 23.5 months (range 11-111), 7 patients developed an ANCA associated vasculitis, while other 3 developed other rheumatic diseases.Finally, when compared with IPF, ILD-MPO patients had a better survival (81.2%±0.9 vs 54.7±0.7 for ILD-MPO and IPF, respectively; p=0.045)Conclusion:ILD positive for anti-MPO antibodies are still a not definite condition. We need larger population to identify possible markers for the evolution in an ANCA associated vasculitis, to define the prognosis of disease and the better therapeutic approach.References: :[1]Mohammad AJ, et al. Pulmonary Involvement in Antineutrophil Cytoplasmic Antibodies (ANCA)-associated Vasculitis: The Influence of ANCA Subtype. J Rheumatol. 2017;44:1458-67Table.Serological, clinical and radiological features of anti-MPO + interstitial lung diseaseNumber30Males/female15/15Median age (years + IQR)68 (17)Median follow-up (months + IQR)39.5 (61)Smoke36.70%ILD pattern Usual interstitial pneumonia63.30% Nonspecific interstitial pneumonia16.70% Hipersensitivity pneumonia10% Other fibrosing pneumonia10%Median FVC (% + IQR)83 (23)Median DLCO (% + IQR)53 (28)Clinical features Raynaud’s phenomenon7.70% Sicca syndrome0 Arthralgias20% Arthritis3.40%Serology Antinuclear antibodies30.80% Anti-extractable nuclear antibodies (ENA)8% Anti-SSA4% Rheumatoid factor21.40%Therapy Immusuppressants23.30% Anti-fibrotic drugs33.30%Disclosure of Interests:None declared

Idiopathic Pulmonary Fibrosis

Chest Imaging ◽  
2019 ◽  
pp. 453-457
Author(s):  
Cylen Javidan-Nejad
Keyword(s):  
Lung Cancer ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Pulmonary Edema ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Usual Interstitial Pneumonia ◽  
Primary Lung Cancer ◽  
Interstitial Lung Diseases ◽  
Pathologic Diagnosis

Idiopathic pulmonary fibrosis (IPF) represents one of the most common chronic interstitial lung diseases. Usual interstitial pneumonia (UIP) is the pathologic diagnosis of IPF and can be diagnosed when honeycombing is present with a basilar and peripheral predominance and findings not typical of UIP are absent. In the current era, when a diagnosis of UIP is made with confidence on HRCT, biopsy can be avoided. Yet, one must be familiar with mimics of UIP/IPF (most notably pulmonary edema superimposed on emphysema) to avoid confusion misdiagnosis. Radiologists must also be familiar with potential complications of UIP including progression, infection, accelerated fibrosis (which can be lethal) and primary lung cancer (which has an increased incidence in UIP).

scholarly journals Probable usual interstitial pneumonia pattern on chest CT: is it sufficient for a diagnosis of idiopathic pulmonary fibrosis?

2020 ◽  
Vol 55 (4) ◽  
pp. 1802465 ◽  
Author(s):  
Jun Fukihara ◽  
Yasuhiro Kondoh ◽  
Kevin K. Brown ◽  
Tomoki Kimura ◽  
Kensuke Kataoka ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Survival Time ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Final Diagnosis ◽  
Chest Ct ◽  
Hazard Ratio ◽  
Adjusted Hazard Ratio ◽  
Made In

Recent studies have suggested that in patients with an idiopathic interstitial pneumonia (IIP), a probable usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT) is sufficient to diagnose idiopathic pulmonary fibrosis (IPF) without histopathology.We retrospectively compared the prognosis and time to first acute exacerbation (AE) in IIP patients with a UIP and a probable UIP pattern on initial chest CT.One hundred and sixty IIP patients with a UIP pattern and 242 with a probable UIP pattern were identified. Probable UIP pattern was independently associated with longer survival time (adjusted hazard ratio 0.713, 95% CI 0.536–0.950; p=0.021) and time to first AE (adjusted hazard ratio 0.580, 95% CI 0.389–0.866; p=0.008). In subjects with a probable UIP pattern who underwent surgical lung biopsy, the probability of a histopathological UIP pattern was 83%. After multidisciplinary discussion and the inclusion of longitudinal behaviour, a diagnosis of IPF was made in 66% of cases. In IPF patients, survival time and time to first AE were not associated with CT pattern. Among subjects with a probable UIP pattern, compared to non-IPF patients, survival time and time to first AE were shorter in IPF patients.In conclusion, IIP patients with a probable UIP pattern on initial chest CT had a better prognosis and longer time to first AE than those with a UIP pattern. However, when baseline data and longitudinal behaviour provided a final diagnosis of IPF, CT pattern was not associated with these outcomes. This suggests diagnostic heterogeneity among patients with a probable UIP pattern.

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