6MWT: 6-minute walk test; ANA: antinuclear antibody; CCP: citric citrullinated peptide; CTD: connective tissue disease; CTD-ILD: connective tissue disease-associated interstitial lung disease; dsDNA: double-stranded DNA; ENA: extractable nuclear antigens; HRCT: high-resolution computed tomography; IIP: idiopathic interstitial pneumonia; ILD: interstitial lung disease; IPAF: interstitial pneumonia with autoimmune features; RNA: ribonucleic acid; SRP: signal recognition particle.

PRACTICE AID

Access the activity, “Recognizing and Managing Progressive Fibrosing ILD in Patients With Connective Tissue Diseases: Examining the Potential Clinical Implications of Antifibrotic Therapy,” at PeerView.com/ZBA40.

A Possible Approach to CTD Evaluation

History• Joint pain, stiffness,

or swelling• Skin thickening or

tightening• Rash in sun-exposed areas• Dryness of the eyes

or mouth• Raynaud’s• Heartburn/regurgitation• Family history of CTD

Physical Exam• Joints• Skin• Hands

Clinical Suspicion of ILD

Multidisciplinary meeting

Consensus diagnosis

IPAF IIPCTD-ILD

Standard InvestigationHistory, physical exam (with detailed rheumatologic assessment),

lung function testing, 6MWT, echo, HRCT ± biopsy

Antibody TestingCore tests: ANA, ENA (including anti-Scl70, SSA/Ro, SSB/La, RNP, Sm, Jo-1),

rheumatoid factor and anti-CCP, anti-dsDNA

Additional/desirable autoantibodies: Scleroderma associated (anti-Th/To, RNA polymerase, PM/Scl) and extended myositis panel (anti t-RNA synthetase,

including PL-7, PL-12; Mi-2; SRP; CADM140/MDA5)

Continual reassessment and

surveillance for secondary causes of ILD

HRCT in CTD-ILD1,2

CTD-ILD: connective tissue disease-associated interstitial lung disease; HRCT: high-resolution computed tomography; NSIP: nonspecific interstitial pneumonia; SSc-ILD: systemic sclerosis–associated interstitial lung disease; UIP: usual interstitial pneumonia.1. Chung JH. Am J Radiol. 2018;210. 2. Castelino FV et al. Arthritis Res Ther. 2010;12:213.

PRACTICE AID

Access the activity, “Recognizing and Managing Progressive Fibrosing ILD in Patients With Connective Tissue Diseases: Examining the Potential Clinical Implications of Antifibrotic Therapy,” at PeerView.com/ZBA40.

Ground-glass opacities (red arrow) and traction bronchiectasis suggestive of NSIP

in SSc-ILD

Honeycombing (blue arrows) with ground-glass opacities suggestive of UIP

in SSc-ILD

ILD: interstitial lung disease; SSc: systemic sclerosis.1. Saketkoo LA et al. J Rheumatol. 2014;41:792-798.

Access the activity, “Recognizing and Managing Progressive Fibrosing ILD in Patients With Connective Tissue Diseases: Examining the Potential Clinical Implications of Antifibrotic Therapy,” at PeerView.com/ZBA40.

Questions to Help Assess Dyspnea and Cough in Patients With SSc1

PRACTICE AID

SSc-ILD Clinical Pearls

• Screening for lung involvement is an essential and aggressive element of caring for people with SSc

• Time lost in detecting lung involvement is lung lost

• Normal pulmonary function testing does not exclude progressive ILD; look for downward trends in pulmonary function tests and hallmark symptoms

• It is essential to aggressively inquire about symptoms

• Maintain a high index of suspicion, obtaining a high-resolution, noncontrasted CT of chest in high-risk patients and those with symptoms

• Treating lung involvement demands intense attention to controlling reflux and sinus symptoms, maximizing nutrition and other aspects of physical function, such as pulmonary rehabilitation and physical conditioning

Do you cough? Is it a drycough?

Do you cough when taking adeep breath in?

Do you cough with laughing orsneezing?

Do you cough while talking?

Does coughing make you feelmore short of breath?

Do you notice it takes youlonger to do the following?

– Mow the lawn – Vacuum or mop – Make the bed

Are you able to keep up withyour peers/spouse/friendswhen walking?

Do you feel your peers/spouse/friends slow theirpace because of you?

Do you feel that bending overtakes your breath away?

Cough Dyspnea