Identification and Diagnosis Chapter 2: Clinical Clues

Watch Chapter 3 here.

Hello, and welcome to this program. 

In this session, we’ll discuss how to identify interstitial lung disease, or ILD, in patients with systemic autoimmune rheumatic disease, or SARD. These conditions include rheumatoid arthritis, systemic sclerosis, inflammatory myopathy or myositis, mixed connective tissue disease, and Sjögren disease. 

The goal today is to walk through the diagnostic pathway— pulmonary function testing, or PFTs, high-resolution computed tomography, or HRCT, and multidisciplinary discussion—and to show how this plays out in a patient scenario. 

To bring this to life, I want to introduce you to Maria. 

Maria is a 48-year-old woman with seropositive rheumatoid arthritis, or RA. Recently, she’s noticed something new: progressive exertional shortness of breath and a persistent dry cough. These symptoms have been present for several months. They’re nonspecific, and they haven’t gone away. 

Today, we’ll keep coming back to Maria as our guide. She represents many patients we see in the clinic—someone with well-controlled joint disease who suddenly develops respiratory symptoms. 

So, what are the red flags?  

The key warning signs for any ILD, including SARD, are exertional dyspnea, a persistent dry cough, bibasilar fine inspiratory crackles upon auscultation, and asthenia. Some patients may not have symptoms due to limited mobility, such as joint synovitis and damage, or may have different reasons for such symptoms, including heart failure.  

Here is where Maria gives us teaching value. She already has two of these: exertional dyspnea and cough. We may hear fine crackles if we examine her carefully and listen at the lung bases with a stethoscope.  

Fine inspiratory crackles are not subtle — they’re a sensitive, reproducible marker of early interstitial lung disease. In fact, in a prospective cohort of 290 patients, 93% of patients with idiopathic pulmonary fibrosis and more than 70% of those with other ILDs had fine crackles at presentation, often before PFTs or radiology were abnormal. 

For Maria, if we do hear basilar crackles, even with normal resting oxygen saturation, that is a powerful red flag. It tells us we need to escalate the workup.  

Recent studies have tested pragmatic checklists of red flags in RA. A cohort of  107 patients from Italy validated that dyspnea, cough, and crackles—alone and in combination—are significantly associated with RA-ILD. In their data, dyspnea alone had a sensitivity of 63% and specificity of 60%. Crackles were even more sensitive, around 67%. The combination of dyspnea or crackles gave the best overall accuracy, about 63%. 

Therefore, when Maria shows up with dyspnea and dry cough, and potentially crackles, that’s exactly the profile that warrants an evaluation for ILD. 

Risk isn’t evenly distributed. We know that older age, male sex, smoking history, high disease activity scores, and certain autoantibodies such as anti-cyclic citrullinated peptide and RF increase the probability of RA-associated ILD. 

Maria is 48, female, and seropositive. She’s not in the highest-risk demographic, but she has persistent symptoms.  

In Maria’s case, both her RA profile and her therapy history need to be weighed carefully. 

Thank you for watching. 

We’ve followed Maria’s story from her first nonspecific symptoms through the red flags, risk stratification, diagnostic testing, and multidisciplinary review. 

Maria’s case illustrates the broader lesson: early suspicion, structured evaluation, and collaborative care make the difference. 

If you adopt this pathway in your clinic — screen, test, image, and discuss early — you’ll help patients like Maria avoid delays and receive the care they need. 

For more resources, please visit the Rheumatology and Arthritis Learning Network. 

6MWT – Six-minute walk test 
ANA – Antinuclear antibody 
Anti-CCP – Anti–cyclic citrullinated peptide antibody 
Anti-Ro52 – Anti-Ro/SSA autoantibody 
CTD-ILD – Connective tissue disease–associated interstitial lung disease 
DLCO – Diffusing capacity of the lungs for carbon monoxide 
FVC – Forced vital capacity 
GGO – Ground-glass opacity 
HRCT – High-resolution computed tomography 
IIM – Inflammatory idiopathic myopathy / inflammatory myositis 
ILD – Interstitial lung disease 
IPF – Idiopathic pulmonary fibrosis 
L – Liters 
LIP – Lymphocytic interstitial pneumonia 
MCTD – Mixed connective tissue disease 
MDD – Multidisciplinary discussion 
MDT – Multidisciplinary team 
NSIP – Nonspecific interstitial pneumonia 
OP – Organizing pneumonia 
PFT – Pulmonary function test 
PPF – Progressive pulmonary fibrosis 
RA – Rheumatoid arthritis 
RF – Rheumatoid factor 
SARD – Systemic autoimmune rheumatic disease 
SSc – Systemic sclerosis 
SS – Sjögren disease 
sec – Seconds 
UIP – Usual interstitial pneumonia 
VA – Alveolar volume 

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