Characteristics and risk factors of interstitial pneumonia with autoimmune features.

BACKGROUND: Interstitial pneumonia with autoimmune features (IPAF) has features of connective tissue disease-associated interstitial lung disease (CTD-ILD), but without meeting criteria for a specific CTD. We compared baseline characteristics, survival, and response to treatment of IPAF to both CTD-ILD and unclassifiable ILD. METHODS: Measurements were extracted from a prospective registry. Baseline features and survival were compared in IPAF against both CTD-ILD and unclassifiable ILD. Linear trajectory of lung function decline (%-predicted forced vital capacity [FVC%] and diffusion capacity of the lung for carbon monoxide [DLCO%]) before and after initiation of mycophenolate or azathioprine were compared in IPAF against both CTD-ILD and unclassifiable ILD using linear mixed models. RESULTS: Compared to CTD-ILD (n = 1240), patients with IPAF (n = 128) were older, more frequently male, and had greater smoking history. Compared to unclassifiable ILD (n = 665), patients with IPAF were younger, more frequently female, and had worse baseline lung function. IPAF had higher mortality compared to CTD-ILD and similar risk of mortality compared to unclassifiable ILD. Mycophenolate initiation was associated with stabilization of FVC% and DLCO% in all ILD subtypes except for FVC% in patients with IPAF, and azathioprine initiation with stabilization of FVC% and DLCO% in all ILD subtypes except for FVC% decline in IPAF and DLCO% decline in CTD-ILD. CONCLUSION: Patients with IPAF had worse survival compared to those with CTD-ILD and similar mortality to unclassifiable ILD, with treatment being associated with stabilization in lung function in all three ILDs. It is uncertain whether IPAF should be considered a distinct ILD diagnostic subgroup.

Systemic sclerosis associated interstitial lung disease: a conceptual framework for subclinical, clinical and progressive disease.

OBJECTIVES: To establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD). METHODS: A conceptual framework for subclinical, clinical and progressive ILD was provided to 83 experts, asking them to use the framework and classify actual SSc-ILD patients. Each patient profile was designed to be classified by at least four experts in terms of severity and risk of progression at baseline; progression was based on 1-year follow-up data. A consensus was reached if ≥75% of experts agreed. Experts provided information on which items were important in determining classification. RESULTS: Forty-four experts (53%) completed the survey. Consensus was achieved on the dimensions of severity (75%, 60 of 80 profiles), risk of progression (71%, 57 of 80 profiles) and progressive ILD (60%, 24 of 40 profiles). For profiles achieving consensus, most were classified as clinical ILD (92%), low risk (54%) and stable (71%). Severity and disease progression overlapped in terms of framework items that were most influential in classifying patients (forced vital capacity, extent of lung involvement on high resolution chest CT [HRCT]); risk of progression was influenced primarily by disease duration. CONCLUSIONS: Using our proposed conceptual framework, international experts were able to achieve a consensus on classifying SSc-ILD patients along the dimensions of disease severity, risk of progression and progression over time. Experts rely on similar items when classifying disease severity and progression: a combination of spirometry and gas exchange and quantitative HRCT.

The increasing trend and the seasonal variation in attendance of diffuse parenchymal lung disease patients presenting to a pulmonary clinic in Eastern India.

BACKGROUND: Diffuse parenchymal lung disease (DPLD) is not an uncommon problem in clinical practice. Although the exact prevalence of DPLD in India is not known, the relative etiological distribution in DPLD in India has been reported. There has been no information as regards the seasonality of the disease. PATIENTS AND METHODS: The archive of the Institute of Pulmocare and Research, Kolkata, was searched for the number of new patients registered at the outpatient department to a single consultant (practicing in the same style on appointment only) over years from 2009 to 2019. The attendance (absolute and relative) was arranged year wise and then month wise to look for the annual and seasonal trends, if any. RESULTS: A total of 2226 patients were registered from 2009 to 2019. There has been a steady increase in both the absolute number (104 in 2009 to 204 in 2019) and the relative percentage of attendance (4.36% in 2009 to 6.9% in 2019) of new registration of DPLD patients over the years. Regarding seasonal variation, two consistent peaks in attendance have been observed as December-January and April-May over the years with dips in February and September; the first being more consistent then the latter. CONCLUSIONS: The increase in relative attendance in the DPLD patients over the years needs further investigation to establish a rising trends in incidence and prevalence of DPLD. The unequivocal trend in seasonal variation needs attention and further research.

Diagnosis and Management of Fibrotic Interstitial Lung Diseases.

Nonidiopathic pulmonary fibrosis (non-IPF) progressive fibrotic interstitial lung diseases (PF-ILDs) are a heterogeneous group of ILDs, often challenging to diagnose, although an accurate diagnosis has significant implications for both treatment and prognosis. A subgroup of these patients experiences progressive deterioration in lung function, physical performance, and quality of life after conventional therapy. Risk factors for ILD progression include older age, lower baseline pulmonary function, and a usual interstitial pneumonia pattern. Management of non-IPF P-ILD is both pharmacologic and nonpharmacologic. Antifibrotic drugs, originally approved for IPF, have been considered in patients with other fibrotic ILD subtypes, with favorable results in clinical trials.

Comparative analysis of connective tissue disease-associated interstitial lung disease and idiopathic pulmonary fibrosis from a tertiary care centre in Pakistan.

OBJECTIVE: To compare the characteristics of connective tissue disease-associated interstitial lung disease with idiopathic pulmonary fibrosis at a tertiary care hospital. METHODS: The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised demographical, clinical and radiological data of patients with interstitial lung disease between October 2016 and October 2017 accessed through the outpatient data registry. Data was compared in terms of characteristics and key features of patients with connective tissue disease-associated interstitial lung disease with those of idiopathic pulmonary fibrosis. Statistical analysis was done using STATA 12. RESULTS: Of the 184 patients, 52(29.3%) had connective tissue disease-associated interstitial lung disease and 62(35%) had idiopathic pulmonary fibrosis. The most prevalent conditions among connective tissue disease-associated interstitial lung disease patients were rheumatoid arthritis 22(42.3%) and scleroderma 13(25%). Compared to patients with idiopathic pulmonary fibrosis, those with connective tissue disease-associated interstitial lung disease were predominantly younger (p<0.001) and female (p<0.001). History of gastroesophageal reflux disease was also significantly lower in connective tissue disease-associated interstitial lung disease (p=0.05). CONCLUSIONS: Connective tissue disease-associated interstitial lung disease patients were found to be younger and predominantly female compared to patients of idiopathic pulmonary fibrosis.

Connective tissue disease-associated interstitial lung disease and outcomes after hospitalization: A cohort study.

BACKGROUND: The impact of hospitalization on patient outcomes is increasingly recognized and considered in the prognostication of many pulmonary disorders. We sought to evaluate the impact of hospitalization on survival in connective tissue disease-interstitial lung disease (CTD-ILD) patients. METHODS: A chart review of patients with CTD-ILD followed at a tertiary care center was performed. Patients were stratified into two groups based on hospitalization status. Outcomes of the groups were compared using Kaplan-Meier survival analyses as well as multivariate competing risk analysis. RESULTS: There were 137 patients identified with confirmed CTD-ILD. Patients who underwent hospitalization for any reason had a significant decrease in transplant-free survival compared to the never hospitalized cohort (3-year survival 60% vs. 94%; p = 0.0001). Hospitalization for ≥7 days was associated with worse outcomes than those hospitalized for <7 days (median survival 1.59 years vs. 7.17 years, p = 0.0012). Based on multivariate competing risk analysis, factors associated with death, with lung transplantation as a competing risk, were age (HR = 1.05 [95% 1.01-1.09]; P = 0.0443), male gender (HR = 4.94 [95% CI: 1.58-15.41]; P = 0.006), and all cause hospitalization (HR = 11.97 [95% CI: 1.36-105.49]; P = 0.0253). CONCLUSION: This study highlights the impact of hospitalization on subsequent outcomes in the CTD-ILD population with a significantly reduced transplant-free survival demonstrated, especially after cardiopulmonary hospitalization events.

Utility of autoimmune serology testing in the assessment of uncharacterized interstitial lung disease: a large retrospective cohort review.

BACKGROUND: Autoimmune serologies are often obtained in the initial evaluation of uncharacterized interstitial lung disease (ILD). Whether this practice is helpful in delineating connective-tissue disease related ILD (CTD-ILD) is not well known. We assessed the frequency of incident CTD-ILD as detected by autoimmune serology testing and presenting clinical signs and symptoms. METHODS: Consecutive patients seen at our institution over a four year period with newly diagnosed uncharacterized ILD and autoimmune serologic testing were included. Serologic assessment was performed as a standardized order set of 13 laboratory tests. Presenting demographics and clinical signs or symptoms suggestive of autoimmune disease were correlated with the presence or absence of positive serology studies and final CTD-ILD diagnoses. RESULTS: Overall prevalence of newly diagnosed CTD-ILD was 6.9% (42 of 605). Positive serology was seen in 35.2% (213 of 605) of screened ILD. CTD-ILD was diagnosed in 19.2% of those with positive serology, and 52.8% of those with both positive serology and suggestive clinical signs or symptoms. Only 1.4% of those with positive serology and negative review of systems were diagnosed with CTD-ILD. CTD-ILD diagnoses were made more frequently in younger patients ≤60 years with no diagnoses made after the age of 80 (P = 0.009). Positive serology in non-CTD-ILD cases did not appear to confer any survival advantage. CONCLUSIONS: The yield of autoimmune serology testing in uncharacterized ILD appears greatest in those with suggestive clinical signs or symptoms on presentation for CTD-ILD.