Connective Tissue Disease-related Interstitial Lung Research Articles

The Spectrum of Interstitial Lung Disease in Connective Tissue Disease.

Interstitial lung disease (ILD) is a common cause of morbidity and mortality in patients with connective tissue disease (CTD). In a minority of patients the ILD may be the presenting (or only) manifestation of an underlying CTD. Diagnosis of CTD-related ILD relies on a multidisciplinary team including pulmonologists, pathologists, radiologists, and rheumatologists, as the imaging and pathologic findings may be indistinguishable from idiopathic interstitial pneumonias. Moreover, many patients with ILD are suspected of having an underlying CTD but do not meet all of the necessary criteria for a specific disorder. This article provides a pattern-based approach to the imaging of CTD-related ILD and also reviews relevant clinical, pathologic, and serologic data that radiologists should be familiar with as part of a multidisciplinary team.

Connective tissue disease-related interstitial lung disease.

Interstitial lung disease (ILD) is commonly present in patients with an underlying connective tissue disease (CTD), particularly those with systemic sclerosis, rheumatoid arthritis, and inflammatory myositis. The clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. Distinguishing features in the clinical, radiographic, and histopathologic characteristics of CTD-ILD subsets can predict prognosis and treatment response. Treatment often consists of combinations of immunosuppressive medications, but there is a paucity of guidance in the literature to help clinicians determine appropriate screening and management of CTD-ILD. As such, there is a critical need for studies that can elucidate the natural history of the CTD-ILD, as well as clarify optimal therapies for CTD patients with ILD.

Benefit of adjunctive tacrolimus in connective tissue disease-interstitial lung disease

We evaluated the safety and effectiveness of adjunctive tacrolimus therapy with conventional immunosuppression in patients with severe connective tissue disease-related interstitial lung disease (CTD-ILD).We included patients from our interstitial lung disease (ILD) registry with CTD-ILD, in whom tacrolimus was added to corticosteroids and an additional immunosuppressive agent. Demographic data, clinical features, lung function, radiographic images, and pathologic findings were reviewed. Effectiveness was assessed by comparing pulmonary function tests (PFTs) closest to tacrolimus initiation to PFTs approximately 6–12 months later. Corticosteroid dose at these time points was also evaluated. We report adverse events attributed to tacrolimus.Seventeen patients with CTD-ILD were included in adverse event analysis; twelve were included in efficacy analysis. Length of tacrolimus therapy ranged from 6 to 110 months (mean 38.8 months ± 31.4). The mean improvement in percent predicted total lung capacity was 7.5% ± 11.7 (p = 0.02). Forced vital capacity mean improvement was 7.4% ± 12.5 (p = 0.06). The average decrease in corticosteroid dose at follow-up was 20.3 mg ± 25.2 (p = 0.02) with complete discontinuation in six patients. No patients experienced a life-threatening adverse event attributed to tacrolimus.Tacrolimus can be effective and is well tolerated as an adjunctive therapy and allows tapering of corticosteroids.

Patient Perspectives in OMERACT Provide an Anchor for Future Metric Development and Improved Approaches to Healthcare Delivery in Connective Tissue Disease Related Interstitial Lung Disease (CTD-ILD).

The impact and natural history of connective tissue disease related interstitial lung disease (CTD-ILD) are poorly understood; and have not been previously described from the patient's perspective. This investigation sought insight into CTD-ILD from the patients' perspective to add to our knowledge of CTD-ILD, identify disease-specific areas of unmet need and gather potentially meaningful information towards development of disease-specific patient-reported outcome measures (PROMs). A mixed methods design incorporating patient focus groups (FGs) querying disease progression and life impact followed by questionnaires with items of importance generated by >250 ILD specialists were implemented among CTD-ILD patients with rheumatoid arthritis, idiopathic inflammatory myopathies, systemic sclerosis, and other CTD subtypes. FG data were analyzed through inductive analysis with five independent analysts, including a patient research partner. Questionnaires were analyzed through Fisher's Exact tests and hierarchal cluster analysis. Six multicenter FGs included 45 patients. Biophysiologic themes were cough and dyspnea, both pervasively impacting health related quality of life (HRQoL). Language indicating dyspnea was unexpected, unique and contextual. Psycho-social themes were Living with Uncertainty, Struggle over Self-Identity, and Self-Efficacy - with education and clinician communication strongly emphasised. All questionnaire items were rated 'moderately' to 'extremely' important with 10 items of highest importance identified by cluster analysis. Patients with CTD-ILD informed our understanding of symptoms and impact on HRQoL. Cough and dyspnea are central to the CTD-ILD experience. Initial FGs have provided disease-specific content, context and language essential for reliable PROM development with questionnaires adding value in recognition of patients' concerns.

The evaluation of clinical usefulness of transbrochoscopic lung biopsy in undefined interstitial lung diseases: a retrospective study.

Previous studies mostly focused on the diagnostic accuracy of transbronchoscopic lung biopsy (TBLB) in the diagnosis of interstitial lung diseases (ILDs). We aimed to explore the clinical usefulness of TBLB results in the diagnostic procedure of undefined ILDs. The retrospective analysis included patients undergoing TBLB for the diagnosis of undefined ILDs from January 2007 to December 2010. The clinically useful TBLB was defined as that lead to a specific histopathological diagnosis or that was consistent with the working diagnosis based on existing clinical and radiological data. A total of 664 patients were included in the study. TBLB failed to obtain lung parenchyma in 155 cases (23.3%). TBLB was considered clinically helpful in 202 procedures (30.4%), including 114 cases that provided definitive histopathological diagnoses and 88 cases that were consistent with working diagnoses. Among 202 cases of clinically useful TBLBs, the majority were diagnosed as pulmonary alveolar proteinosis (PAP) (67 cases, 33.2%), connective tissue disease-related ILDs (CTD-ILDs) (65, 32.2%) and idiopathic pulmonary fibrosis (IPF) (33, 16.3%). Although TBLB could provide definitive histopathological diagnoses in all cases diagnosed as PAP, only few cases of IPF (7, 21.2% of IPF diagnoses) and CTD-ILDs (9, 13.8% of CTD-ILD diagnoses) could be identified by TBLBs. The clinical usefulness of TBLB, in conjunction with thorough clinical and radiological data, in the diagnosis of ILDs may be varied depending on different subtypes. The use of histopathological analysis and the type of biopsy employed should therefore be considered on a case-by-case basis.

Clinical significance of mTOR, ZEB1, ROCK1 expression in lung tissues of pulmonary fibrosis patients.

BackgroundIdiopathic pulmonary fibrosis (IPF) is a fatal lung disease of unknown causes. Three proteins (mammalian target of rapamycin, mTOR; zinc finger E-box-binding homeobox 1, ZEB1; Rho-associated, coiled-coil containing protein kinase 1, ROCK1) may be related to pulmonary fibrosis. However, they have not been assessed in human pulmonary fibrosis. We assessed the clinical significance of mTOR, ZEB1, and ROCK1 expression in human pulmonary fibrosis of usual interstitial pneumonia (UIP) pattern.MethodsThe mTOR, ZEB1, and ROCK1 expression was evaluated by immunohistochemical staining of 30 surgical lung biopsy tissues from 26 IPF and 4 UIP pattern connective tissue disease related interstitial lung disease (CTD-ILD) patients. The expression scores correlated with the clinical features.ResultsThe mTOR, ZEB1 and ROCK1 mainly expressed in alveolar epithelial cells of UIP lungs. The histological fibrosis scores and lung function decline in the strong mTOR expression group were higher than those in the weak and intermediate expression group. Patients with positive ZEB1 expression had higher fibrosis scores and greater decline in carbon monoxide diffusion capacity (DLCO) than patients with negative ZEB1 expression. Patients with positive mTOR or ZEB1 expression had poorer prognosis than that of patients with negative mTOR or ZEB1 expression, although it was not statistically significant. ROCK1 was not associated with the studied clinicopathological features.ConclusionsThe mTOR and ZEB1 expression in pulmonary fibrosis patients significantly correlated with the fibrosis score and lung function decline, indicating that it may be related to the prognosis of pulmonary fibrosis. Further studies involving large numbers of homogeneous IPF patients are warranted.

The relative frequencies of causes of widespread ground-glass opacity: A retrospective cohort

PurposeThe purpose of our study was to determine the relative frequencies of causes of widespread ground-glass opacity (GGO) in an unselected, consecutive patient population and to identify any associated imaging findings that can narrow or reorganize the differential. Materials and methodsThe study was approved by the center's IRB and is HIPPA compliant. Cases with widespread GGO in the radiology report were identified by searching the Radiology Information System. Medical records and CT scan examinations were reviewed for the causes of widespread GGO. Associations between a less dominant imaging finding and a particular diagnosis were analyzed with the chi square test. Our study group consisted of 234 examinations with 124 women and 110 men and a mean age of 53.7 years. ResultsA cause was established in 204 (87.2%) cases. Hydrostatic pulmonary edema was most common with 131 cases (56%). Interstitial lung diseases (ILD) were the next most common, most often hypersensitivity pneumonitis (HP) (n=12, 5%) and connective tissue disease related ILD (n=7, 3%). Infection accounted for 5% (12 cases). A few miscellaneous diseases accounted for 5 cases (2.1%). The combination of septal thickening and pleural effusions had a specificity of 0.91 for hydrostatic pulmonary edema (P<.001) while centrilobular nodules and air trapping had a specificity of 1.0 for HP. In 24 (10.2%) patients, increased opacification from expiration was incorrectly interpreted as representing widespread ground glass opacity.The relative frequency of disease dramatically changed according to the setting. In the inpatient setting, diffuse alveolar disease and diffuse infection accounted for all of the known diagnoses. Pulmonary edema accounted for 75% of the diagnoses and diffuse infection accounted for approximately 7%. In the outpatient setting, interstitial lung disease was the most common cause, accounting for 26 of 53 cases (49%).Regarding immunocompromised patients, hydrostatic pulmonary edema was still the most common cause (46%) with diffuse infection (24%) the next most likely diagnosis. For patients with bone marrow suppression, 80% of the cases were due to opportunistic viral infection. ConclusionWidespread GGO is most commonly a manifestation of hydrostatic pulmonary edema. Outpatients will most often have HP or connective tissue disease related ILD. Associated findings are helpful for the diagnosis of hydrostatic pulmonary edema and HP.

Prevalence of Venous Thromboembolic Events and Diagnostic Performance of the Wells Score and Revised Geneva Scores for Pulmonary Embolism in Patients with Interstitial Lung Disease: A Prospective Study

To determine the prevalence of venous thromboembolism (VTE) in patients with connective tissue disease-related interstitial lung diseases (CTD-ILD) and idiopathic interstitial pneumonia (IIP) and to further evaluate associated risk factors. To also examine the diagnostic performance of the Wells score and the revised Geneva scores for diagnosing pulmonary embolism (PE) in ILD patients. Fifty-seven patients with CTD-ILD and IIP were prospectively enrolled. Plasma D-dimer was measured by ELISA. Deep-vein thrombosis (DVT) was examined by venous ultrasonography and PE by computed tomography pulmonary angiography. PE prevalence was further assessed by the Wells score and the revised Geneva score. VTE was diagnosed in 15 (26.3%, 15/57) patients. Bivariate analysis revealed that dyspnoea (OR 3.750, 95%CI 1.095-12.842, P=0.035), lower extremity oedema (OR 8.667, 95%CI1.814-41.408, P=0.007), palpitations (OR 4.75, 95%CI1.073-21.032, P=0.040), and positive D-dimer (OR 5.087, 95%CI 1.015-25.485, P=0.048) were associated with VTE. Using the Wells Score, 46 (80.70%), eight (14.4%) and three (5.26%) patients had a low, intermediate and high probability of PE, respectively, with seven (15.22%), three (37.5%) and two (66.67%) of the respective cases confirmed. By the revised Geneva score, 23 (40.35%), 32 (56.14%) and two (3.51%) patients had a low, intermediate and high probability of PE, respectively, with two (8.70%), nine (28.13%) and one (50.00%) of the respective cases confirmed. The AUC for the Wells score and the revised Geneva score was 0.720±0.083 (CI 0.586 to 0.831) and 0.704± 0.081 (CI 0.568 to 0.817), respectively. VTE can be seen in approximately one fourth of patients with CTD-ILD or IIP and the Wells score and the revised Geneva score can be used for categorising PE risk.

Reconciling healthcare professional and patient perspectives in the development of disease activity and response criteria in connective tissue disease-related interstitial lung diseases.

Interstitial lung diseases (ILD), including those related to connective tissue disease (CTD), and idiopathic pulmonary fibrosis (IPF) carry high morbidity and mortality. Great efforts are under way to develop and investigate meaningful treatments in the context of clinical trials. However, efforts have been challenged by a lack of validated outcome measures and by inconsistent use of measures in clinical trials. Lack of consensus has fragmented effective use of strategies in CTD-ILD and IPF, with a history of resultant difficulties in obtaining agency approval of treatment interventions. Until recently, the patient perspective to determine domains and outcome measures in CTD-ILD and IPF had never been applied. Efforts described here demonstrate unequivocally the value and influence of patient involvement on core set development. Regarding CTD-ILD, this is the first OMERACT working group to directly address a manifestation/comorbidity of a rheumatic disease (ILD) as well as a disease not considered rheumatic (IPF). The OMERACT 11 proceedings of the CTD-ILD Working Group describe the forward and lateral process to include both the medical and patient perspectives in the urgently needed identification of a core set of preliminary domains and outcome measures in CTD-ILD and IPF.

Safety and Efficacy of Tacrolimus in Connective Tissue Disease-Related Interstitial Lung Disease

Safety and Efficacy of Tacrolimus in Connective Tissue Disease-Related Interstitial Lung Disease

A cohort study of interstitial lung diseases in central Denmark

Interstitial lung diseases (ILDs) form a heterogeneous group of diseases with varying degrees of inflammation and fibrosis. Epidemiological data based on the current diagnostic criteria are sparse. To characterize the incidence rate of ILDs and idiopathic pulmonary fibrosis (IPF) in Danish patients diagnosed at a referral hospital, to evaluate disease severity and survival in these ILD patients and to compare the use of the 2001 and 2011 guidelines to diagnosis of IPF. Single-centre, retrospective, observational cohort study including incident patients diagnosed with ILD at Aarhus University Hospital between 2003 and 2009. All diagnoses were re-evaluated according to current diagnostic criteria. Disease severity in IPF was assessed using the GAP index. The ILD incidence was 4.1 per 100,000 inhabitants/year. IPF was the most common diagnosis (28%) followed by connective tissue disease-related ILD (14%), hypersensitivity pneumonitis (7%) and non-specific interstitial pneumonia (NSIP) (7%). The GAP index was a strong predictor of survival in IPF. Twenty-three patients who had IPF based on the 2001 criteria had a "possible UIP" HRCT pattern but no lung biopsy, and IPF could therefore not be diagnosed based on the 2011 criteria. ILD and IPF incidence was 4.1 and 1.3 per 100,000 inhabitants/year. The diagnostic re-evaluation raised the number of IPF diagnoses, but a diagnostic "grey zone" was still evident in patients with UIP features not qualifying the patients to be diagnosed with IPF. The GAP index was valuable as a measure of IPF severity in this cohort.

Effect of Rituximab in Patients with Cyclophosphamide-non-responsive Connective Tissue Disease-Related Interstitial Lung Disease: A Case Series Study

Objective: To report our experience of intravenous cyclophosphamide pulse therapy and the effect of rituximab (RTX) therapy in patients with cyclophosphamide-non-responsive connective tissue disease associated interstitial lung disease (CTD-ILD).Methods: We retrospectively evaluated the medical records of patients with CTD-ILD. All patients received intravenous cyclophosphamide pulse (IVCYC) therapy. The patients who received IVCYC+RTX were non-responsive to IVCYC treatment. At 0 to 6 months prior to the treatment, these patients underwent a pulmonary function test (PFT). High resolution computed tomography was performed before the initiation of treatment.Results: Ten patients were enrolled in this study. A significant percentage of patients showed stabilization or improvement of pulmonary function after treatment. The response rates of the IVCYC and IVCYC+RTX groups were 50% (5/10) and 60% (3/5), respectively.Conclusions: Patients with CTD-ILD who received IVCYC with or without RTX therapy showed a significant improvement in lung function. RTX therapy could be employed as a salvage therapy and combined with IVCYC early for refractory CTD-ILD.

AB0815 Living with connective tissue disease related interstitial lung disease: Patient experiences of the disease over time

BackgroundVery limited information is available about the patient experience of connective tissue disease-related interstitial lung disease (CTD-ILD), in terms of global effects on functioning in various psychological and social spheres...

THU0486 Peroxisome Proliferator Activated Receptorγ Anti-Fibrosis Mechanism in Connective Tissue Disease Related Interstitial Lung Disease

BackgroundConnective tissue disease (CTD) is a disease with a high incidence and a poor therapeutic effect. In recent years, in vitro and in vivo experimental studies have confirmed that the...

The diagnosis efficacy and safety of video-assisted thoracoscopy surgery (VATS) in undefined interstitial lung diseases: a retrospective study.

To evaluate the efficacy and safety of lung biopsies by video-assisted thoracoscopy surgery (VATS) in the diagnosis of undefined interstitial lung disease (ILD). The retrospective analysis was performed in 32 who patients underwent VATS for the diagnosed with ILD from Jan 2007 to Dec 2011. The main reason for VATS for all the patients was due to no specific diagnosis could be obtained after non-invasive methods, transbronchial lung biopsy (TBLB) examination and the consultation with pulmonologist, radiologist and pathologist. The clinical profiles, chest high resolution computerized tomography (HRCT), laboratory profile, TBLB as well as the diagnosis of before and after the VATS were analyzed. The surgery site, biopsy number, duration of the thoracic drain, post-operative complications were also recorded. The 30- and 90-day post-operative mortality rates were calculated. The risk factors associated with the incidence of post-operative complications were assessed. The specific diagnosis could be established in all patients after VATS lung biopsies, with change from previous ones in 27 (84.4%). Among 20 cases (62.5%) diagnosed as unclassified ILD before the surgery, 14 (70.0%) were diagnosed as nonspecific interstitial pneumonia (NSIP), 3 (15.0%) as idiopathic pulmonary fibrosis (IPF) and 3 (15.0%) as connective tissue disease-related ILD (CTD-ILD). Among the 7 cases with complete change of diagnosis after VATS, 4 (57.1%) were cryptogenic organizing pneumonia (COP). The number of site of biopsy had no significant impact on the diagnostic efficacy. There were no significant change of vital sign and lung function after the VATS. 21 (65.6%) patients had post-operative complications, including pulmonary infection (56.3%), pulmonary atelectasis (28.1%) and pneumothorax (25.0%). The 30- and 90-day mortality rates were 0 and 5.2% respectively. Patients were divided into 2 groups based on the incidence of post-operative complications, and no significant difference was found in regards to the age, body mass index (BMI), smoking index, lung function, anesthesia method, duration of remaining the thoracic drain and the use of immunosuppressive drugs or steroids. VATS is a safe and effective procedure for the diagnosis of ILD which were unclassified after routine evaluation, transbronchial lung biopsy and consultation with pulmonologist, radiologist and pathologist.

Autoimmune-Featured Interstitial Lung Disease: A Distinct Entity

Patients with interstitial lung disease (ILD) may have features of an autoimmune disorder that do not meet the diagnostic criteria for connective tissue diseases. We determined the prevalence and characteristics of autoimmune-featured ILD (AIF-ILD) and compared these with those of idiopathic pulmonary fibrosis (IPF) and known connective tissue disease-related ILD (CTD-ILD). Patients with ILD who did not meet the criteria for a connective tissue disease were defined as having AIF-ILD if they had a sign or symptom suggestive of a connective tissue disease and a serologic test reflective of an autoimmune process. Clinical characteristics, high-resolution CT images, and lung biopsy specimens were analyzed and compared with those of patients with IPF and CTD-ILD. Survival was evaluated using a Kaplan-Meier curve. Two hundred subjects completed the questionnaire and serologic testing. AIF-ILD was identified in 32%, IPF in 29%, and CTD-ILD in 19%. Gender, age, and race differed among groups (P < .01). Sixty-two percent of patients with AIF-ILD had a typical usual interstitial pneumonia (UIP) pattern on CT images. In 31 patients with AIF-ILD, lung biopsy specimens showed UIP in 81% and nonspecific interstitial pneumonia in 6%. Patients with AIF-ILD and IPF had similar survival, worse than those with CTD-ILD (P < .01). Antinuclear antibody (ANA) titers ≥ 1:1280 were associated with improved survival in patients with AIF-ILD (P = .02). Systematic evaluation of symptoms and serologic tests in ILD can identify AIF-ILD. A UIP pattern on CT images and histopathology is common in AIF-ILD. Although survival for patients with AIF-ILD is poor, ANA titers ≥ 1:1280 are associated with improved survival.

Developing Disease Activity and Response Criteria in Connective Tissue Disease-related Interstitial Lung Disease

The interstitial lung diseases (ILD) are a group of heterogeneous diseases that exert significant morbidity and mortality in connective tissue diseases (CTD). There is no consensus on measures of disease activity or therapeutic responsiveness, which hampers effective drug development and regulatory evaluation of candidate therapies. The CTD-ILD Special Interest Group represents an international multidisciplinary effort to identify consensus on criteria to measure disease activity and therapeutic response in CTD-ILD. We summarize the design of the studies we are conducting and progress leading to the OMERACT 10 and 2010 EULAR meetings.

Local apoptosis promotes collagen production by monocyte-derived cells in transforming growth factor β1-induced lung fibrosis.

BackgroundCollagen-containing leukocytes (CD45+Col-I+) accumulate in diseased and fibrotic tissues. However, the precise identity of these cells and whether injury is required for their recruitment remain unknown. Using a murine model of pulmonary fibrosis in which an inducible, bioactive form of the human transforming growth factor (TGF)-β1 gene is targeted to the lung, we characterized the cell surface phenotype of collagen-containing CD45+ cells in the lung and tested the hypothesis that apoptotic cell death responses are essential to the accumulation of CD45+Col-I+ cells.ResultsOur studies demonstrate that CD45+Col-I+ cells appearing in the TGF-β1-exposed murine lung express markers of the monocyte lineage. Inhibition of apoptosis via pharmacological caspase blockade led to a significant reduction in CD45+Col-I+ cells, which appear to accumulate independently of alternatively activated macrophages. There are also increased levels of apoptosis and greater numbers of CD45+Col-I+ in the lung tissue of patients with two distinct forms of fibrotic lung disease, idiopathic pulmonary fibrosis and connective tissue disease-related interstitial lung disease, when compared to lung from healthy normal controls. These findings are accompanied by an increase in collagen production in cultured monocytes obtained from subjects with fibrotic lung disease. Treatment of these cultured cells with the caspase inhibitor carbobenzoxy-valyl-alanyl-aspartyl-[O-methyl]-fluoromethylketone (Z-VAD/fmk) reduces both apoptosis and collagen production in all subjects.ConclusionsInterventions that prevent collagen production by monocytes via modulation of caspase activation and of apoptosis may be ameliorative in monocyte-associated, TGF-β1-driven processes such as pulmonary fibrosis.

Pneumorrhachis and pneumomediastinum in connective tissue disease-related interstitial lung disease: case series from a tertiary care teaching hospital in South India

Pneumomediastinum has been described as a rare complication of connective tissue diseases. Here, we report four cases of pneumomediastinum: three of which are associated with dermatomyositis and one with mixed connective tissue disease. All our patients had interstitial lung disease. The first case of dermatomyositis described below was complicated by epidural emphysema (pneumorrhachis) in addition to pneumomediastinum. Pneumorrhachis is reported in many isolated case reports and series in the setting of asthma, pneumothorax, blunt chest trauma, etc. Less than 10% of pneumomediastinum cases develop this complication and vast majority of cases resolve spontaneously. The mechanism behind this has been postulated to be the passage of air through the intervertebral foramen. Others suggest entrapment of air which dissects between paraspinal soft tissues and along the vascular and nerve sheaths into the epidural space. This is the first ever reported case of epidural emphysema in connective tissue disease to the best of our knowledge.

The impact of rheumatological evaluation in the management of patients with interstitial lung disease

We hypothesized that multi-disciplinary assessment of patients with interstitial lung disease (ILD) would lead to improved diagnosis and management. Our multi-disciplinary ILD group evaluated 50 patients referred to our tertiary care practice over a 12-month period. Evaluation consisted of independent assessments by a pulmonologist and rheumatologist, including complete history and physical examination, review of laboratory data, as well as review of imaging and pathological specimens in conjunction with a radiologist and pathologist experienced in ILD. Therapy was initiated or changed in collaboration with the referring physicians. Mean age was 65 years for patients with rheumatic or CTD-related ILD (CTD-ILD) and 70 years for those with idiopathic pulmonary fibrosis (IPF). The final diagnosis after evaluation in the clinic was 25 patients with CTD-ILD, 15 patients with IPF and 10 patients with other forms of lung disease. Of the patients with a final diagnosis of CTD-ILD, 28% were referred with a diagnosis of IPF. Among those referred with CTD-ILD, 36% had their diagnosis changed to an alternate CTD-ILD. In total, the diagnosis was changed in 54% of patients who presented to the ILD clinic. Changes in therapy occurred in 80% of patients with CTD-ILD and in 27% of patients with IPF. A multi-disciplinary ILD clinic offers patients an innovative mode of health-care delivery that can enhance diagnosis, affect treatment regimens and improve quality of care. Rheumatologists play a significant role in the clinical care of these patients and should evaluate all patients with ILD.