scholarly journals A rare case of Interstitial lung disease complicated by overlapping syndrome with obstructive ventilatory dysfunction as the first symptom

2021 ◽  
Vol 3 (4) ◽  
pp. 1-5
Author(s):  
Yuling LIANG ◽  
Yan LEI ◽  
Jiaqi XU ◽  
Liqi HUANG ◽  
Lian LIN ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Cyclic Peptide ◽  
Interstitial Fibrosis ◽  
High Titer ◽  
High Resolution Computed Tomography ◽  
Respiratory Dysfunction ◽  
The Past ◽  
Pulmonary Interstitial Fibrosis ◽  
Function Test

Interstitial lung disease is often a serious complication of connective tissue disease（CTD）, representing the first cause of death in CTD. However, there are few reports of SSc-RA overlap-associated interstitial pneumonia. Respiratory dysfunction as the first clinical manifestation is even rarely reported. We herein described a case of a male patient who developed significant respiratory dysfunction as the principal clinical sign for the past 6 months, plus newly developed skin thickening in bilateral upper limbs and pain and swelling of multiple joints for the past 6 weeks. Extensive immunological screening showed high titer of antinuclear antibodies(ANA), rheumatoid factor(RF), anti-cyclic peptide containing citrulline (anti-CCP)，and positive anti-Scl-70 antibody. Chest high resolution computed tomography(HRCT) was performed and hence ILD was confirmed. Pulmonary function test (PFT) revealed obstructive ventilatory dysfunction rather than restrictive ventilatory dysfunction. So bronchodilation test was performed and asthma was considered. Finally, after a multidisciplinary team (MDT) discussion, the diagnosis of asthma and ILD associated with SSc-RA overlap was established. It is important to note that CTD associated pulmonary interstitial fibrosis must be considered as the differential diagnosis of any newly diagnosed interstitial pulmonary disease. In addition, if patient with dyspnea is highly suspicious of pulmonary interstitial fibrosis, bronchodilation test is still needed to exclude asthma in order to avoid misdiagnosis.

scholarly journals Lung Manifestation of Collagen Vascular Disease in HRCT

2020 ◽  
Vol 8 (1) ◽  
pp. 9-13
Author(s):  
Jagruti Kalola ◽  
Anjana Trivedi ◽  
Hiral Happani ◽  
Mohit Chauhan
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Fibrosis ◽  
Airway Disease ◽  
Ground Glass ◽  
Connective Tissue Disorders ◽  
High Resolution Computed Tomography ◽  
Presenting Symptoms ◽  
Disease Pattern

Background: The aim of this paper was to evaluate the thoracic manifestations associated with the Connective tissue disorders, with an emphasis on interstitial and airway disease pattern on the High Resolution computed tomography (HRCT) findings. Subjects and Methods: The present study was conducted for a period of one year. A total of 50 patients with various connective tissue disorders having respiratory complaints were evaluated. Results:  During the study period 50 patients (80%females and 20%males) underwent evaluation. Cough and dyspnea were  the most common presenting symptoms. Variety of thoracic abnormalities weredetected in 67 (95%) cases. Most common abnormality detected on HRCT was interstitial fibrosis/interstitial lung disease present in (60%) cases. Most common parenchymal abnormalities seen were reticulations (61.4%), ground glass opacification (40%), mosaic attenuation (32.8%) and honeycombing (24.3%). Airway abnormalities seen were bronchiectasis (48.5%), emphysema (12.8%), and ground glass nodules (2.8%). Conclusion: Interstitial lung disease is the most common pulmonary manifestation among patients with connective tissue disorders, and early detection and prompt treatment is expected to improve the outcome.

scholarly journals Risk factors for pulmonary interstitial fibrosis in patients with systemic sclerosis

2020 ◽  
Author(s):  
Ji Yang ◽  
Xiangxiang Cui ◽  
Ming Li
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Fibrosis ◽  
Heart Function ◽  
Digital Ulcers ◽  
Double Positive ◽  
High Resolution Computed Tomography ◽  
Clinical Indicators ◽  
Pulmonary Interstitial Fibrosis ◽  
Function Test ◽  
Single Positive

Abstract Background Pulmonary interstitial fibrosis (PIF) is a frequent manifestation of systemic sclerosis (SSc). However, there is a lack of good clinical indicators for predicting PIF. Here, we evaluated autoantibodies and clinical phenotypes to predict the involvement of PIF in SSc. Methods Peripheral blood was collected from the 86 SSc patients enrolled in this study to detect autoantibodies. PIF, lung function, and heart function were analyzed by lung high-resolution computed tomography, pulmonary function test, and standard transthoracic echocardiography, respectively. The correlation between autoantibodies, clinical phenotype, and internal organ involvement were summarized. Results PIF occurred in 68.4% anti-SCL-70 antibody positive SSc patients (p = 0.0045), 59.8% anti-centromere antibody negative SSc patients (p = 0.0013), and 71.4% anti-SSA antibody positive SSc patients (p = 0.0107). PIF occurred in 94.1% anti-SCL-70 antibody and anti-SSA antibody double positive SSc patients, which was higher than in anti-SCL-70 antibody single positive patients, p = 0.0452. More SSc patients with digital ulcers had PIF (73.5%) than SSc patients without digital ulcers (p = 0.0008). Conclusion These data suggest that SSc patients with double positive for anti-SCL-70 and anti-SSA antibodies are more prone to develop PIF. In addition, digital ulcers are an important and convenient predictor for PIF in SSc patients.

Increased Serum B Cell Activating Factor and a Proliferation-inducing Ligand Are Associated with Interstitial Lung Disease in Patients with Juvenile Dermatomyositis

2015 ◽  
Vol 42 (12) ◽  
pp. 2412-2418 ◽  
Author(s):  
Norimoto Kobayashi ◽  
Ichiro Kobayashi ◽  
Masaaki Mori ◽  
Shinji Sato ◽  
Naomi Iwata ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
B Cell ◽  
Juvenile Dermatomyositis ◽  
High Titer ◽  
Serum Levels ◽  
Interleukin 18 ◽  
Fatal Complication ◽  
High Resolution Computed Tomography ◽  
B Cell Activating Factor

Objective.Rapidly progressive interstitial lung disease (RP-ILD) is an intractable and fatal complication of juvenile dermatomyositis (JDM). This study evaluated serum levels of B cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL) in JDM patients with complicating ILD, and their association with ILD phenotypes, clinical variables, and anti-melanoma differentiation-associated gene 5 (MDA5).Methods.We measured the levels of BAFF, APRIL, and anti-MDA5 in the sera of 23 JDM patients with ILD [8 in the RP-ILD group and 15 in the chronic ILD (C-ILD) group], 17 JDM patients without ILD (non-ILD group), and 10 age-matched controls, using the ELISA method. ILD was identified by high-resolution computed tomography.Results.Serum BAFF titers were significantly higher in the JDM patients with RP-ILD versus those with C-ILD (p = 0.011) and in healthy controls (p = 0.0004). The C-ILD group had significantly higher levels of BAFF versus controls (p ≤ 0.0001). Serum APRIL was markedly elevated in the RP-ILD group as compared with the C-ILD group (p = 0.003) and controls (p = 0.006). In patients with ILD, both BAFF and APRIL levels were correlated with serum Krebs von den Lungen-6 and interleukin 18. Subjects with high titer anti-MDA5 (> 200 U) had higher levels of BAFF and APRIL than those with low titer anti-MDA5 (< 100 U; p = 0.019 and p = 0.0029, respectively), which may have been due to a relationship between RP-ILD and high anti-MDA5 titer.Conclusion.Our findings of markedly elevated levels of BAFF and APRIL in patients with RP-ILD JDM suggest the potential importance of these cytokines in the diagnosis and treatment of RP-ILD accompanying JDM.

scholarly journals AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1598.2-1599
Author(s):  
I. Rusu ◽  
L. Muntean ◽  
M. M. Tamas ◽  
I. Felea ◽  
L. Damian ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
High Resolution Computed Tomography ◽  
Hrct Patterns

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared

scholarly journals Interstitial Lung Disease and Anti-Myeloperoxidase Antibodies: Not a Simple Association

2021 ◽  
Vol 10 (12) ◽  
pp. 2548
Author(s):  
Marco Sebastiani ◽  
Fabrizio Luppi ◽  
Gianluca Sambataro ◽  
Diego Castillo Villegas ◽  
Stefania Cerri ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Rheumatic Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Usual Interstitial Pneumonia ◽  
Clinical History ◽  
High Resolution Computed Tomography ◽  
Function Testing ◽  
And Diffusion

Anti-neutrophil cytoplasmic antibodies (ANCA), mainly anti-myeloperoxidase (MPO) antibodies, have been frequently identified in patients with idiopathic pulmonary fibrosis (IPF). However, their role remains unclear, and only 7–23% of these patients develops clinically overt vasculitis. We aimed to investigate the clinical, serological, and radiological features and prognosis of anti-MPO-positive interstitial lung disease (ILD) patients. Fifty-eight consecutive patients firstly referred for idiopathic interstitial pneumonia and showing serological positivity of anti-MPO antibodies were retrospectively enrolled. For each patient, clinical data, lung function testing, chest high-resolution computed tomography (HRCT) pattern, and survival were recorded. Thirteen patients developed a rheumatic disease during a median follow-up of 39 months. Usual interstitial pneumonia (UIP) was the most frequent ILD pattern, significantly influencing the patients’ survival. In fact, while the 52-week survival of the overall population was 71.4 ± 7.5%, significantly higher than IPF, survivals of anti-MPO patients with UIP pattern and IPF were similar. Forced vital capacity and diffusion lung capacity for CO significantly declined in 37.7 and 41.5% of cases, respectively, while disease progression at chest HRCT was observed in 45.2%. A careful clinical history and evaluation should always be performed in ILD patients with anti-MPO antibodies to quickly identify patients who are developing a systemic rheumatic disease.

scholarly journals Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study)

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Mouhamad Nasser ◽  
Sophie Larrieu ◽  
Loic Boussel ◽  
Salim Si-Mohamed ◽  
Fabienne Bazin ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Healthcare Resource ◽  
Pulmonary Function Tests ◽  
Healthcare Services ◽  
Resource Utilisation ◽  
High Resolution Computed Tomography ◽  
Healthcare Database ◽  
Healthcare Resource Utilisation ◽  
Kaplan Meier

Abstract Background There is a paucity of data on the epidemiology, survival estimates and healthcare resource utilisation and associated costs of patients with progressive fibrosing interstitial lung disease (PF-ILD) in France. An algorithm for extracting claims data was developed to indirectly identify and describe patients with PF-ILD in the French national administrative healthcare database. Methods The French healthcare database, the Système National des Données de Santé (SNDS), includes data related to ambulatory care, hospitalisations and death for 98.8% of the population. In this study, algorithms based on age, diagnosis and healthcare consumption were created to identify adult patients with PF-ILD other than idiopathic pulmonary fibrosis between 2010 and 2017. Incidence, prevalence, survival estimates, clinical features and healthcare resource usage and costs were described among patients with PF-ILD. Results We identified a total of 14,413 patients with PF-ILD. Almost half of them (48.1%) were female and the mean (± standard deviation) age was 68.4 (± 15.0) years. Between 2010 and 2017, the estimated incidence of PF-ILD ranged from 4.0 to 4.7/100,000 person-years and the estimated prevalence from 6.6 to 19.4/100,000 persons. The main diagnostic categories represented were exposure-related ILD other than hypersensitivity pneumonitis (n = 3486; 24.2%), idiopathic interstitial pneumonia (n = 3113; 21.6%) and rheumatoid arthritis-associated ILD (n = 2521; 17.5%). Median overall survival using Kaplan–Meier estimation was 3.7 years from the start of progression. During the study, 95.2% of patients had ≥ 1 hospitalisation for respiratory care and 34.3% were hospitalised in an intensive care unit. The median (interquartile range) total specific cost per patient during the follow-up period was €25,613 (10,622–54,287) and the median annual cost per patient was €18,362 (6856–52,026), of which €11,784 (3003–42,097) was related to hospitalisations. Limitations included the retrospective design and identification of cases through an algorithm in the absence of chest high-resolution computed tomography scans and pulmonary function tests. Conclusions This large, real-world, longitudinal study provides important insights into the characteristics, epidemiology and healthcare resource utilisation and costs associated with PF-ILD in France using a comprehensive and exhaustive database, and provides vital evidence that PF-ILD represents a high burden on both patients and healthcare services. Trial registration ClinicalTrials.gov, NCT03858842. ISRCTN, ISRCTN12345678. Registered 3 January 2019—Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT03858842

AB0796 THE EVALUATION OF LUNG DIAGNOSTIC PROCEDURE AT THE ONSET OF INFLAMMATORY RHEUMATIC DISEASES WITH INTERSTITIAL LUNG DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1422.3-1423
Author(s):  
T. Hoffmann ◽  
P. Oelzner ◽  
F. Marcus ◽  
M. Förster ◽  
J. Böttcher ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Interstitial Lung Disease ◽  
Lung Function ◽  
Lung Disease ◽  
Sensitivity And Specificity ◽  
Lung Function Test ◽  
Inflammatory Rheumatic Diseases ◽  
X Ray ◽  
Function Test ◽  
Chest X Ray

Background:Interstitial lung disease (ILD) in inflammatory rheumatic diseases (IRD) is associated with increased mortality. Moreover, the lung is one of the most effected organs on IRD. Consequently, screening methods were required to the detect ILD in IRD.Objectives:The objective of the following study is to evaluate the diagnostic value of lung function test, chest x-ray and HR-CT of the lung in the detection of ILD at the onset of IRD.Methods:The study is designed as a case-control study and includes 126 patients with a newly diagnosed IRD. It was matched by gender, age and the performance of lung function test and chest x-ray. The sensitivity and specificity were verified by crosstabs and receiver operating characteristic (ROC) curve analysis. The study cohort was divided in two groups (ILD group: n = 63 and control group: n = 63). If possible, all patients received a lung function test and optional a chest x-ray. Patients with pathological findings in the screening tests (chest x-ray or reduced diffusing capacity for carbon monoxide (DLCO) < 80 %) maintained a high-resolution computer tomography (HR-CT) of the lung. Additionally, an immunological bronchioalveolar lavage was performed in the ILD group as gold standard for the detection of ILD.Results:The DLCO (< 80 %) revealed a sensitivity of 83.6 % and specificity of 45.8 % for the detection of ILD. Other examined parameter of lung function test showed no sufficient sensitivity as screening test (FVC = Forced Vital Capacity, FEV1 = Forced Expiratory Volume in 1 second, TLC = Total Lung Capacity, TLCO = Transfer factor of the Lung for carbon monoxide). Also, a combination of different parameter did not increase the sensitivity. The sensitivity and specificity of chest x-ray for the verification of ILD was 64.2 % versus 73.6 %. The combination of DLCO (< 80 %) and chest x-ray showed a sensitivity with 95.2 % and specificity with 38.7 %. The highest sensitivity (95.2 %) and specificity (77.4 %) was observed for the combination of DLCO (< 80 %) and HR-CT of the lung.Conclusion:The study highlighted that a reduced DLCO in lung function test is associated with a lung involvement in IRD. DLCO represented a potential screening parameter for lung manifestation in IRD. Especially patients with suspected vasculitis should receive an additional chest x-ray. Based on the high sensitivity of DLCO in combination with chest x-ray or HR-CT for the detection of ILD in IRD, all patients with a reduced DLCO (< 80%) should obtained an imaging of the lung.Disclosure of Interests:None declared

scholarly journals POS0842 LUNG ULTRASOUND TO ASSESS THE SEVERITY OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC SCLEROSIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 675.1-675
Author(s):  
C. Bruni ◽  
L. Mattolini ◽  
L. Tofani ◽  
L. Gargani ◽  
N. Landini ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Quantitative Methods ◽  
Lung Ultrasound ◽  
Significant Negative Correlation ◽  
High Resolution Computed Tomography ◽  
Quantitative Score ◽  
Quantitative Scoring ◽  
B Lines

Background:Interstitial lung disease (ILD) is one of the most common complications and one of the main causes of morbidity and mortality in Systemic Sclerosis (SSc). High-resolution computed tomography (HRCT) is the gold standard for the diagnosis of ILD and it allows its quantification. Among semi-quantitative methods, Goh et al proposed a semi-quantitative scoring system to visually quantify ILD extent, with categorical cut-off of 20% to distinguish limited and extensive parenchymal involvement with prognostic implications. More recently, the use of radiomics has allowed the objective quantification of ILD through the use of dedicated software, which calculate different parameters of lung density.Given the exposure to ionizing radiation that the procedure entails, other methods of ILD evaluation are being studied, among which lung ultrasound (LUS) identifies the B-lines as a main feature of ILD. So far, different evidences have proposed the use of LUS for the screening of ILD, even in the early phases of the disease and in subclinical lung involvement.Objectives:the aim of this study is to test the role of LUS in quantifying the severity of SSc-ILD, evaluated with both semi-quantitative visual radiological and quantitative radiomic scores.Methods:Adult SSc patients classified according to the ACR/EULAR 2013 criteria patients were assessed with pulmonary function test (PFTs), lung ultrasound and HRCT over 60 days. CT images were analysed qualitatively (by presence/absence of ILD), semi-quantitatively (categorical Goh score <20% vs> 20% of extent and the continuous extent Goh score made from 5 levels’ assessment– 0 to 100%) and quantitatively [with the densitometric radiomic data obtained through the Horos software - Mean lung attenuation (MLA), Standard Deviation (SD), Kurtosis, Skewness and Lung volume (LV)]. LUS was used to quantify the B-lines detected in each patient by scanning a total of 13 intercostal spaces, on both anterior and posterior chest wall.Results:Among 59 SSc patients (81% women, mean age 48±14 years, 45% anti-Scl70 positive), 23 (39%) presented ILD on HRCT, of which 14 limited and 9 extensive. The mean visual semi-quantitative score was 6%, ranging from 0 to 66%. Our data showed a significantly different number of B-Lines in ILD vs non-ILD patients (median 38 vs 9, p <.005), a result which was further confirmed among non-ILD vs ILD> 20% (median 47 vs 9, p=.001) and ILD <20% (median 36 vs 9, p=.001) patients. Conversely, the number of B-lines was not statistically different between patients with ILD <20% and >20% (median 47 vs 36, p=.78). We observed a significant negative correlation between the number of B-lines and FVC (r=-.472, p<.05) TLC (r=-.436, p=.003), DLco (r=-.515, p<.001), DLCO/VA (r=.-306, p=.03). Finally, the number of B-lines showed a statistically significant correlation with the Goh score on 5 levels (r=.437, p=.001), MLA (r=.571, p<.001), kurtosis (r=-.285, p=.028), skewness (r=-.370, p = .004) and LV (r=-.277, p=.033). All data were confirmed analysing anterior and posterior B-Lines separately.Conclusion:Our study confirms that LUS represents a useful tool for the identification of SSc-ILD. In addition, we showed that LUS may be useful also for the quantification of the severity of SSc-ILD, by correlating with PFT parameters, radiomics parameters and visual radiological evaluation. Together with the PFTs, LUS could be used to increase the accuracy of the screening and, potentially, of the follow-up of SSc-ILD patients.Disclosure of Interests:Cosimo Bruni: None declared, Lavinia Mattolini: None declared, Lorenzo Tofani: None declared, Luna Gargani Consultant of: GE Healthcare, Philips Healthcare and Caption Health, Nicholas Landini: None declared, Gemma Lepri: None declared, Martina Orlandi: None declared, Serena Guiducci: None declared, Silvia Bellando Randone: None declared, Marco Matucci-Cerinic: None declared

scholarly journals Evaluation of poor prognostic factors of respiratory related death in microscopic polyangiitis complicated by interstitial lung disease

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Shogo Matsuda ◽  
Takuya Kotani ◽  
Takayasu Suzuka ◽  
Takao Kiboshi ◽  
Keisuke Fukui ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Laboratory ◽  
Microscopic Polyangiitis ◽  
Survival Rates ◽  
Lower Lobe ◽  
Left Lower Lobe ◽  
Fibrosis Score ◽  
High Resolution Computed Tomography

AbstractThe prognosis of microscopic polyangiitis (MPA) with interstitial lung disease (ILD) is significantly worse than that of MPA without ILD. However, the clinical characteristics in MPA-ILD, especially poor prognostic factors, are not elucidated. We evaluated demographic, clinical, laboratory, and radiological findings, treatments, and outcomes of 80 patients with MPA, and investigated prognostic factors of respiratory-related death in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positive MPA-ILD. Ground-glass opacity and fibrosis were evaluated as scores on high-resolution computed tomography (HRCT). The presence of ILD was consistent with a high risk of respiratory-related death (hazard ratio, 4.8; P = 0.04). Multivariable logistic regression analyses using propensity scoring showed right or left lower lobe fibrosis score to be significantly associated with respiratory-related death (P = 0.0005 and 0.0045, respectively). A right or left lower lobe fibrosis score ≥ 2, indicating the presence of honeycombing at 1 cm above the diaphragm, was determined to be the best cut-off value indicating a poor prognosis. The 5-year survival rate was significantly lower in patients with right or left lower lobe fibrosis score ≥ 2 (survival rates: 37% and 19%, respectively) than those with a score < 2 (71% and 68%, respectively) (P = 0.002 and 0.0007, respectively). These findings suggest that the presence of honeycomb lesions in bilateral lower lobes on chest HRCT was associated with respiratory-related death in patients with MPO-ANCA positive MPA-ILD.

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