Related Experiment Video
Updated: Jun 24, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Ascertainment of collagen vascular disease in patients presenting with interstitial lung disease
Shikha Mittoo1, Allan C Gelber, Lisa Christopher-Stine
1Division of Rheumatology, University of Manitoba, Winnipeg Rheumatic Disease Unit, RR149 Rehabilitation Hospital, Health Sciences Centre, 800 Sherbrook Street, Winnipeg, MB, R3A 1M4, Canada.
Insights
Collagen vascular disease (CVD) is frequently undiagnosed in interstitial lung disease (ILD) patients. High ANA titers and elevated muscle enzymes (CPK or aldolase) indicate a higher likelihood of CVD in ILD referrals.
Area of Science:
- Pulmonology
- Rheumatology
- Internal Medicine
Background:
- Interstitial lung disease (ILD) patient outcomes are affected by co-occurring collagen vascular disease (CVD).
- The prevalence of underlying CVD in ILD patients is not well-established.
- This study aimed to determine the frequency of new CVD diagnoses in ILD referrals.
Purpose of the Study:
- To ascertain the frequency of collagen vascular disease (CVD) in patients referred for interstitial lung disease (ILD).
Main Methods:
- Retrospective analysis of 114 consecutive patients evaluated at a specialized ILD clinic.
- Assessment for the development of collagen vascular disease (CVD) based on established diagnostic criteria.
Main Results:
- Nearly one-third of ILD patients met criteria for CVD.
- 15% of patients received a new CVD diagnosis during their ILD evaluation.
- New CVD diagnoses were associated with younger age, high ANA titers (≥1:640), and elevated creatine phosphokinase (CPK) or aldolase levels.
Conclusions:
- Undiagnosed collagen vascular disease (CVD) is potentially common in interstitial lung disease (ILD) referrals.
- High-titer ANA and elevated CPK or aldolase are significant indicators for CVD in this population.
Introduction:
Previous studies of interstitial lung disease (ILD) suggest that prognosis and therapeutic response are influenced by the presence of underlying collagen vascular disease (CVD). Yet, what proportion of patients presenting with ILD have CVD is largely unknown. We sought to determine the frequency of a new CVD diagnosis in an ILD referral population.
Materials/Patients And Methods:
We retrospectively studied 114 consecutive patients evaluated at the Johns Hopkins Interstitial Lung Disease Clinic for the development of CVD.
Results:
In this retrospective cohort, nearly one-third of the 114 patients with confirmed ILD satisfied published criteria for a CVD diagnosis. Seventeen (15%) patients were diagnosed with a new CVD as a direct consequence of their ILD evaluation. Patients with new CVD diagnosis were younger than those without new CVD diagnosis: 51.4years (95% CI 45-58years) and 60years (95% CI 57-63), respectively (p=0.01). Moreover, an ANA>or=1:640 (p=0.03) and elevated levels of creatine phosphokinase (CPK) or aldolase (p<0.001) were associated with a new CVD diagnosis.
Conclusions:
Unrecognized collagen vascular disease may be more common than previously appreciated among patients referred with ILD. High titer ANA and an elevated CPK or aldolase are associated with a CVD diagnosis in this referral population.
Related Concept Videos
01:27Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History
01:30Radiological Investigation II: MRI and Ventilation Perfusion Scan
Magnetic Resonance Imaging (MRI) and Ventilation Perfusion Scans are two radiological investigations that offer detailed diagnostic images of the body, particularly lung structures.
MRI
MRI uses magnetic fields and radiofrequency signals to distinguish between normal and abnormal tissues. This technology provides a more detailed diagnostic image than CT scans, enabling it to characterize pulmonary nodules, stage bronchogenic carcinoma, and evaluate inflammatory activity in...
01:22Connective Tissue Cell Types
Fat cells (adipocytes), smooth muscle cells (myoblasts), and bone cells (osteoblasts) are some connective tissue cell types. Some immune system cells...
01:13Radiological Investigation III: Pulmonary Angiogram and PET Scan
Pulmonary Angiogram
A Pulmonary Angiogram is an invasive procedure involving injecting a contrast medium through a catheter threaded into the pulmonary artery or the right side of the heart to visualize the pulmonary vasculature. Computed Tomography (CT) scans have mainly replaced this...
01:11Fibril-associated Collagen
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...
01:29Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care