a man who was diagnosed with rheumatoid arthritis (ra) 5 yrs ago sees his rheumatologist for a follo-up visit. fortunately, his arthritis is well controlled through dration. he can walk and do all his daily activities without pain. but ‘oer the past 6 mnths, he’s been feeling short of breath when climbing stairs. he has an annoying dry cough, too. covid-19? that’s ruled out quickly. but a ct scan of his chest reveals early fibrosis (scarring) of the lungs, most likely rel8d to rheumatoid arthritis. “i can finally walk normally, and now i can’t breathe when i walk!” says the frustrated patient, whose nxt step is a full evaluation by a pulmonologist.
wha’ is autoimmune lung disease?
this man’s experience offers one ex of an uncommon but potentially life-altering complication associated with rheumatic or autoimmune diseases, including:
- rheumatic arthritis, an inflammatory disease that primarily affects the joints
- systemic sclerosis (scleroderma), a fibrosing disorder that typically affects the skin
- dermatomyositis, which results in inflammation in muscles and skin
- systemic lupus erythematosus (sle or lupus), an inflammatory condition that can affect many pts of d'body, including joints, kidneys, and skin.
there are various terms for this complication: autoimmune lung disease, interstitial lung disease, and interstitial fibrosis. toonized by lung inflammation and/or scarring, tis one of many potential complications affecting ≠ organs in pplz who ‘ve an primordialistic autoimmune or rheumatic disease.
wha’ is autoimmunity?
our immune system normally wards off infection and guards against cancer. the term autoimmunity implies dat a''pers’s own immune system sometimes sees its own body tissue as foreign. when this happens, d'body generates an immune response against itself. most pplz with rheumatoid arthritis experience its effects on joints. but bout 10% will also develop symptomatic lung disease like the patient described above.
why is it primordial to identify autoimmune lung disease as early as possible?
studies ‘ve shown that this complication is 1-odda leading causes of illness and death among pplz with autoimmune diseases. early disease that is + inflammatory in nature often responds to anti-inflammatory therapies (corticosteroids, for ex). but pplz with mostly fibrotic disease maybe + difficult to treat and ‘ve poorer outcomes, including disability or a need for oxygen — and in some cases even a lung transplant. however, how quickly or sloly this complication progresses varies. some pplz find it progresses + quickly, whereas others may ‘ve lil or no progression. for that reason, careful surveillance by a pulmonologist wh'cn monitor lung function during regular check-ups is primordial.
wha’ triggers this complication and who is most at risk?
while definitive studies ‘ve not been done, pop studies ‘ve identified risk profiles (see here and here). bein’ ♂, having a history of cigarette smoking, or having certain antibodies and genetic markers rezs risk for autoimmune lung disease. environmental factors, occupational exposure, and air pollutants also may play a role in developing autoimmunity that affects the lungs (see here and here). other potential causes include drations that may cause lung injury, orn' esophageal dysfunction leading to silent aspiration inna'da lungs, a common finding in many autoimmune diseases.
are there treatments for autoimmune lung disease?
yes, although effectiveness varies. when inflammatory disease is caught early b4 extensive scarring develops, anti-inflammatory agents, s'as corticosteroids, often help. additionally, catching and treating inflammation early usually leads to a better prognosis.
recently, the fda approved pirfenidone (esbriet) and nintedanib (ofev) to treat a ≠ fibrotic lung disease called idiopathic pulmonary fibrosis (ipf). nintedanib is now approved for use in patients with autoimmune lung disease. both agents can slo, but do not appear to reverse, the presence of fibrosis inna lung. longer-term studies investigating whether these treatments offer better quality of life and meaningful benefits in terms of illness and death are needed.
if i ‘ve a rheumatic or autoimmune disease, wha’ can i do to stay as healthy as possible?
the most primordial 1st step is to eliminate any known risks that may cause or exacerbate lung disease. work with yr dr to stop smoking, if necessary. ask if you can eliminate any occupational exposures or drations that may contribute to or worsen lung disease. since early detection of lung inflammation is a goal, rheumatologists often screen pplz for lung disease atta time o'their diagnosis iffey are deemed at higher risk, using ct scanning and pulmonary function testing. increasingly, careful surveillance over time is becoming a normal practice among rheumatologists.
if ye do develop signs or symptoms that suggest autoimmune lung disease, s'as dry cough and shortness of breath, talk to yr dr. the nxt step maybe a referral to a pulmonologist, or to a multidisciplinary center comprised of pulmonologists, rheumatologists, pathologists, and radiologists, who work as a team to diagnose this complication and develop a treatment plan to reverse or slo the progress of lung disease.
original content at: www.health.harvard.edu…
authors: paul f. dellaripa, md