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PAH diagnosis and management in the context of CTD ILD

d Aurelie Najm, MD, PhD

Pulmonary arterial hypertension (PAH) is a serious complication of connective tissue disorders. Approximately 25% of patients with PAH have an underlying CTD, making CTD-PAH the second most common cause of PAH. During the session on Management of Lung Complications in CTD at APLAR 2025, Prof. Dinesh Khanna and Masataka Kuwana provided an excellent overview of PAH diagnosis and management strategies the context of CTD-ILD.

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ILD damage fibrosis path

Antifibrotics - A New Class of Therapies in Rheumatology?

d Shervin Assassi, MD

Rheumatologists typically manage systemic autoimmune rheumatic diseases (SARD) and associated interstitial lung disease (ILD) with immunosuppressants. However, evidence increasingly supports also the use of antifibrotic agents in this setting.

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ERS/EULAR guidelines for CTD-related interstitial lung disease

d Jack Cush, MD

The European Respiratory Society (ERS) and European Alliance of Associations for Rheumatology (EULAR) have published clinical practice guidelines for the evaluation and management of connective tissue diseases (CTD) associated interstitial lung disease (ILD) in two simultaneous publications in the European Respiratory Journal and Annals of Rheumatic Diseases.

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COLCHICORT Trial

d Jack Cush, MD

The COLCHICORT trial in acute calcium pyrophosphate (CPP) crystal arthritis patients showed that colchicine was inferior to prednisone treatment and that responses were also influenced by other factors.

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Gout: Highlights from APLAR 2025 Dr. Sheila Reyes, The Philippines, reports on highlights and takeaways from lectures on the treatment of gout from the APLAR 2025 Congress in Fukuoka, Japan. https://t.co/Fxd3MBUYrT https://t.co/gKjHlJXttT

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Dr. John Cush RheumNow ( View Tweet)
Addressing Disparities and Unequal Burdens in ILD Interstitial lung diseases (ILDs) represent a complex spectrum of conditions that share one unifying truth: they are almost always serious, progressive, and life-altering. But for many patients, the burden of disease does not https://t.co/Ubh7R7Xb3E
Dr. John Cush RheumNow ( View Tweet)
Study of 111 TAKayasu arteritis pts Rx w/ 74 MMF+MTX vs 37 CYC/AZA. Response rates at 28 & 52 weeks were 58.1% and 55.4% in the MMF+MTX group, respectively, higher than 32.4% at both time points in the CYC/AZA group. https://t.co/wqlOLH0VEA https://t.co/nbLvcyYkbn

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Dr. John Cush RheumNow ( View Tweet)
Long-term data from the 48-wk phase 2 SLEek study of upadacitinib +/- a BTK inhibitor elsubrutinib, in SLE pts, showed both arms effective (little added from BTK), @ 1yr, 127 pts maintained efficacy, GC dose and flare rates https://t.co/FbTHCGZ4pQ https://t.co/cATXVHsiXV

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Dr. John Cush RheumNow ( View Tweet)
#APLAR2025 Prof Peter Taylor RA patients have ⬆️ rates of CKD https://t.co/DZl9qpbLhH

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Dr Gurdeep S Dulay gurdeep_dulay ( View Tweet)
Systemic Sclerosis–Associated ILD Watch Alicia Hinze, MD, share personalized strategies for managing Systemic Sclerosis–Associated ILD. Sponsored By: Boehringer Ingelheim https://t.co/Mxg9P50m7C #ILD #Scleroderma https://t.co/Cmupl7Dgwl

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Dr. John Cush RheumNow ( View Tweet)
Stay sharp in RA, PsA & SpA care. Get the latest breakthroughs + expert insights at RWCS 2026. Don’t miss 'Year in Review: Rheumatology Advances.' Register now: https://t.co/majEsY4tXK https://t.co/fgGniR1OGk

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RWCS RWCSmtg ( View Tweet)
Metanalysis of 7 studies (1979-2023) shows RA pts on vegetarian or vegan diets achieved no improvement in disease activity or physical function, but did have a small significant improvement in pain. Insufficient Data on Fatigue. Overall, low certainty https://t.co/jeh6gN5Byg
Dr. John Cush RheumNow ( View Tweet)
SPRING-SIR Italian registry of systemic sclerosis: 1917 SSc pts, 56% had GI Sx, assoc w/ longer Dz duration, more severe Dz, diffuse SSc, ILD, digital ulcers, telangiectasias, & tobacco. In 211 very early SSc, 41% GI Sxs (most esophageal), w/ puffy fingers, & dyspnea. https://t.co/l2hStwavuu
Dr. John Cush RheumNow ( View Tweet)
Obstructive sleep apnea in USA is expected to rise from 34·3% in 2020 to 46·2%, resulting in 76·6 million OSA cases by 2050. By 2050 there will be more males w/ OSA (~46 milliion) than females (30·4 million), but greater growth in females over the next 30 years. https://t.co/6YZvSusTWf
Dr. John Cush RheumNow ( View Tweet)
Multimorbidity predicts flares in rheumatoid arthritis A study from Olmstead county and the Mayo Clinic shows that multimorbidity and social factors predict higher rheumatoid arthritis (RA) flares and lower remission rates. https://t.co/yGrvDttcEq https://t.co/45LMtvsFor

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Dr. John Cush RheumNow ( View Tweet)
Based on a survey in Japan, resolution of dry mouth was the most important symptom resolution reported by SJD pts. The JCR CPG for SjD suggests RTX for glandular involvement while BEL for extra-glandular involvements. #APLAR25 @RheumNow https://t.co/SsUvQIM9Eu

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sheila RHEUMarampa ( View Tweet)
Is it D2T RA or Clinical inertia? Concept introduced by Dr Kaneko, Japanese Keio RA cohort, followed for 5yrs 5% patients not fitting the D2T RA definition have MDA or HDA The drivers of persistent DA in RA: -overestimation of treatment effectiveness -avoidance of treatment https://t.co/0pkDaUFH4R

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Aurelie Najm AurelieRheumo ( View Tweet)
Interesting data shared by Dr. Sandhya Pulukool on Asian pts with SjD vs. Caucasians: - Younger age of onset except in Japan - Higher female to male ratio @RheumNow #APLAR25 https://t.co/RPM9zI4tVQ

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sheila RHEUMarampa ( View Tweet)
Peter Taylor on Pre-RA: If Pre-RA is too late for interception, shall we start screening general population? We have to remember RA is a RARE disease Pre-test of RA per year per person would be 0.00025% for primary prevention: A needle in a haystack? @RheumNow @APLAR_org https://t.co/LKGtz1P7sl

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Aurelie Najm AurelieRheumo ( View Tweet)

Peter Taylor describing the range of potential benefits of Interception strategies in Pre-RA: A) From Ideal: full interception B) To Good outcome: stratified intervention based on high risk population for delay/interception C) To ?Beneficial: attenuate severity of established RA https://t.co/6mqA9tV7ME

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Aurelie Najm AurelieRheumo ( View Tweet)
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