Screenings if you have systemic sclerosis (scleroderma)

The main guidance

In 2023 the American College of Rheumatology (ACR) and the American College of Chest Physicians (CHEST) published the first guideline they jointly endorsed on screening and monitoring for interstitial lung disease (ILD) in people with systemic sclerosis and four other rheumatic diseases. It says ILD affects more than half of adults with systemic sclerosis and is the leading cause of death and hospital stays in this group. (ACR/CHEST guideline, 2023) The British Society for Rheumatology (BSR) published a guideline for management of systemic sclerosis in 2024 that includes screening for lung disease, pulmonary hypertension, heart involvement and cancer. (BSR guideline, 2024) The 2022 European Society of Cardiology and European Respiratory Society (ESC/ERS) pulmonary hypertension guideline is described here through an open-access 2026 review. (Review of ESC/ERS 2022, 2026) The NHS says people with scleroderma need regular blood pressure checks and other tests to check for problems with their organs. (NHS)

Checks the guidance lists for adults with systemic sclerosis

Check	Looks for	When	Source
Lung function tests (spirometry, lung volumes, gas transfer)	Interstitial lung disease	At diagnosis; yearly rescreening considered by risk; every 3 to 6 months early on if lung disease is found	(BSR guideline, 2024)(ACR/CHEST guideline, 2023)
High-resolution CT of the chest	Interstitial lung disease	At diagnosis; repeated if symptoms or lung tests worsen, or considered after 1 to 3 years	(BSR guideline, 2024)(ACR/CHEST guideline, 2023)
Echocardiogram, NT-proBNP and DETECT tool	Pulmonary arterial hypertension	Yearly	(BSR guideline, 2024)(Review of ESC/ERS 2022, 2026)
ECG, echocardiogram, troponin and NT-proBNP	Primary heart involvement	Yearly, without symptoms	(BSR guideline, 2024)
Blood pressure	Kidney involvement and scleroderma renal crisis	Regularly; self-checks for people at high risk in early disease	(NHS)(MedlinePlus)(BSR guideline, 2024)
Age-based cancer screening	Cancer	Kept up to date; extra baseline checks over 65 or with high-risk features	(BSR guideline, 2024)

Lungs

The BSR guideline says all people with systemic sclerosis should be screened for ILD with a baseline chest HRCT and lung function tests, including spirometry and gas transfer, so that later lung tests can be read more reliably and early ILD is not missed. With confirmed ILD, it says lung function tests should be repeated every 3 to 6 months in the first 3 to 5 years after diagnosis and considered every 6 to 12 months after that. It says HRCT should be repeated if symptoms or lung tests worsen, and that a repeat after 1 to 3 years to compare with the baseline can be considered. (BSR guideline, 2024)

The ACR/CHEST guideline conditionally recommends lung function tests and HRCT for screening, and conditionally recommends against screening with a chest X-ray, a 6-minute walk test or a walking oxygen test. It suggests that most people with systemic sclerosis have screening tests at presentation, and that people at greater risk be considered for yearly lung function tests, with more frequent screening early in the disease for those with high-risk antibodies such as anti-Scl-70. Once ILD is found, it suggests lung function tests every 3 to 6 months in the first year, then less often once stable, and says how often HRCT is repeated depends on symptoms and on radiation exposure. Its frequencies are suggestions, not firm recommendations. (ACR/CHEST guideline, 2023) See spirometry and COPD screening.

Pulmonary hypertension

The BSR guideline says pulmonary hypertension develops in 1 to 2 percent of people with systemic sclerosis per year of follow-up. It says screening for pulmonary arterial hypertension should be done in all people with systemic sclerosis every year, typically with lung function tests, an echocardiogram, an NT-proBNP blood test and the DETECT tool in suitable people. It says DETECT may be used in adults whose gas transfer (DLCO) is under 60 percent of predicted and who have had the disease for more than 3 years, to decide who needs an echocardiogram and right heart catheterization. (BSR guideline, 2024) A 2026 review says the 2022 ESC/ERS guideline recommended screening all people with systemic sclerosis at the first assessment and then yearly, and that it endorses DETECT, a two-step score using findings such as lung function, NT-proBNP, urate and the ECG. (Review of ESC/ERS 2022, 2026)

Heart

The BSR guideline says screening for primary heart involvement in people without symptoms should be done in all people with systemic sclerosis every year, typically with an ECG, an echocardiogram and troponin and NT-proBNP blood tests (or BNP with kidney disease). Where heart involvement is suspected, it lists a heart MRI and a Holter monitor. It says heart MRI screening may be considered in people at higher risk, such as men and people with diffuse skin disease, anti-topoisomerase I antibodies, early disease or ILD. (BSR guideline, 2024) See an ECG at a checkup.

Blood pressure and kidneys

MedlinePlus says blood pressure is checked because systemic sclerosis can narrow small blood vessels in the kidneys, which can lead to high blood pressure and lower kidney function; its listed tests include a metabolic panel with creatinine and a urine test. (MedlinePlus) The BSR guideline says scleroderma renal crisis is generally linked to new, significant high blood pressure, that recognizing who is at risk helps timely diagnosis, and that people at high risk in early-stage disease should be taught to check their own blood pressure. (BSR guideline, 2024) See blood pressure readings and kidney test results.

Cancer screening

The BSR guideline says cancer accounts for about 16 percent of deaths in systemic sclerosis and that the benefit of extra cancer screening is not yet proven. It says clinicians should make sure people are up to date with age-specific cancer screening such as mammography and bowel screening. For people over 65, or with features linked to cancer such as anti-RNA polymerase III antibodies, it lists baseline screening with a breast exam, a lymph node assessment, a FIT stool test and endoscopy if needed, with CT or PET-CT considered case by case. (BSR guideline, 2024) See cancer screenings by age.

When to contact a provider

MedlinePlus, scleroderma, when to contact a medical professional: (MedlinePlus)

Contact your provider if you develop Raynaud phenomenon, progressive thickening of the skin, or trouble swallowing.

Routine age-based screening

These checks are added to, not instead of, the routine screenings for your age and sex; see screenings by age and gender. For rheumatoid arthritis and lupus, see screenings if you have rheumatoid arthritis or lupus.

Where they differ:

Who gets a baseline HRCT. The BSR guideline says all people with systemic sclerosis should have one; the ACR/CHEST guideline suggests screening for most people with systemic sclerosis, tailored to the individual through shared decision-making. (BSR guideline, 2024)(ACR/CHEST guideline, 2023)
Lung function tests with ILD. ACR/CHEST suggests every 3 to 6 months for the first year, then less often; BSR says every 3 to 6 months for the first 3 to 5 years, then every 6 to 12 months. (ACR/CHEST guideline, 2023)(BSR guideline, 2024)
Repeat HRCT. BSR says a repeat after 1 to 3 years can be considered; ACR/CHEST gives no interval and ties it to symptoms and radiation exposure. (BSR guideline, 2024)(ACR/CHEST guideline, 2023)
Heart screening. BSR lists a yearly ECG, echocardiogram and heart blood tests for primary heart involvement as a weaker (grade 2C) recommendation; its yearly pulmonary hypertension screening is a strong (grade 1B) one. (BSR guideline, 2024)

Your doctor decides what your result means for you.

Questions people ask

Should everyone with systemic sclerosis be checked for lung disease?

The 2024 British Society for Rheumatology (BSR) guideline says all people with systemic sclerosis should be screened for interstitial lung disease with a baseline high-resolution CT (HRCT) of the chest and lung function tests including gas transfer. The 2023 ACR/CHEST guideline suggests that most people with systemic sclerosis have screening tests at presentation, and conditionally recommends lung function tests and HRCT. (BSR guideline, 2024) (ACR/CHEST guideline, 2023)

How often are lung function tests repeated?

Without known lung disease, the ACR/CHEST guideline suggests considering yearly lung function tests based on risk factors. With confirmed lung disease, it suggests every 3 to 6 months in the first year, then less often once stable; the BSR guideline says every 3 to 6 months in the first 3 to 5 years, then every 6 to 12 months. (ACR/CHEST guideline, 2023) (BSR guideline, 2024)

Is a chest X-ray enough to screen the lungs?

No, in the ACR/CHEST guideline. It conditionally recommends against screening with a chest X-ray, noting that studies found it picks up only 58 to 64 percent of cases, and also against screening with a 6-minute walk test. (ACR/CHEST guideline, 2023)

How often is pulmonary hypertension checked?

Every year. The BSR guideline says screening for pulmonary arterial hypertension should be done in all people with systemic sclerosis each year, typically with lung function tests, an echocardiogram, an NT-proBNP blood test and, in suitable people, the DETECT tool. A 2026 review describes the 2022 ESC/ERS guideline as recommending screening at the first assessment and then yearly. (BSR guideline, 2024) (Review of ESC/ERS 2022, 2026)

Why is blood pressure watched so closely?

Systemic sclerosis can narrow small blood vessels in the kidneys, which can lead to high blood pressure and lower kidney function, so MedlinePlus says blood pressure is checked. The NHS says regular blood pressure checks are needed. The BSR guideline advises teaching people at high risk of scleroderma renal crisis, in early-stage disease, to check their own blood pressure. (MedlinePlus) (NHS) (BSR guideline, 2024)