‘You’re not alone’: What rheumatologists need to know about ILD, PAH in scleroderma
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Interstitial lung disease and pulmonary arterial hypertension associated with scleroderma often require consultations and co-management with physicians outside of rheumatology.
In these situations, the most important skill for rheumatologists is knowing when to ask for help, according to Janet E. Pope, MD, MPH, FRCPC, a professor of medicine in the division of rheumatology at the University of Western Ontario, Schulich School of Medicine in Ontario.
In recognition of Scleroderma Awareness Month, Healio spoke with Pope to discuss the dangers associated with ILD and PAH, and what rheumatologists need to know when treating patients with scleroderma.
Healio: What are the big problems associated with scleroderma?
Pope: The highest mortality right now in scleroderma is interstitial lung disease and/or pulmonary arterial hypertension. There are other things, such as scleroderma of the heart and renal crisis as a surrogate for other bad things going on, but really, it is the two main lung complications because they are common and do lead to death in many patients with scleroderma.
Healio: How common are ILD and PAH in patients with scleroderma?
Pope: There are complications that are clinically not relevant, such as a mild interstitial lung disease that might not progress. If you looked at ILD and systemic sclerosis, or scleroderma, maybe you find as high as one in four in the limited subset, and one in three in the diffuse, but clinically relevant is about half that. About one in six patients, or 15%, will have progressive lung disease, and many of these people will succumb to it. It’s the same with pulmonary arterial hypertension.
What I mean is proven pulmonary arterial hypertension — proven by right heart catheterization — is from 8% to 15% of patients with scleroderma. Although there are treatments that improve quality of life and prolong life, it is a lethal complication, and eventually, one in six, or 15%, of patients with scleroderma will succumb to that.
Healio: What should rheumatologists be looking for in terms of early warning signs of these complications?
Pope: The ones who are suspected of developing scleroderma, I believe, should be followed. They would be people who, for instance, have a centromere, positive ANA. They may not yet have puffy fingers, but the patients might feel their fingers have changed. They are people that you would follow, but not intervene upon until we have either better treatment studies that say intervening early is important, or at least watching to see if they get the diagnosis or not over time. The real captive audience are the patients you’ve already labeled with systemic sclerosis.
In early disease, we tend to do a workup. Some sites might do a pulmonary function test and an echo, others would do that and, in addition, a chest X-ray and a high-resolution CT scan of the lung. Periodically, we should screen and that screening should be your history. Go with very specific questions detailing what most patients would do in their normal activity. In a physical exam, we are listening for bilateral basal crackles, we’re looking for signs of right-heart failure, elevated jugular venous pressure, positive hepato-jugular reflux and/or pitting edema.
For interstitial lung disease, if you’re seeing things change on the pulmonary function test, you’re going to investigate further. I think what’s most important for rheumatologists that don’t see a lot of scleroderma is to know that you’re not alone. You can seek help from other specialists — experts in pulmonary hypertension would be the pulmonologists or the cardiologists, and the experts in ILD would be the pulmonologists that have declared a further expertise. There are also scleroderma centers that you can refer patients to.
Healio: Is there anything you wish primary care providers were more aware of regarding identifying scleroderma and these complications?
Pope: Primary care physicians do many common things very well. Diabetes, hypertension, hyperlipidemia targets, etc. I don’t think the primary care doctors need to worry other than the ANA is almost always positive and that 95% to 99% have Raynaud’s disease, but they are going to see primary, or idiopathic, Raynaud’s. Most primary care physicians don’t have a practice large enough to have even one patient with scleroderma. I don’t think they should worry about it, but if something is different, the patient needs to be sent to somebody else.
Healio: When should screening for scleroderma and these complications begin?
Pope: Once you make a diagnosis of scleroderma, mixed connective tissue disease or undifferentiated connective tissue disease with scleroderma features, you should start screening for ILD and at least a baseline look for pulmonary hypertension with an echocardiogram, the detect algorithm or some other means to screen. The risk for pulmonary hypertension increases with age of onset and disease duration. It also increases in patients with ILD who get hypoxia.
Otherwise, if someone is a suspect and nothing has happened for a long period of time, I don’t think you have to screen every patient, every year, even though guidelines might suggest annual screenings. Again, you don’t need to be an expert if you’re seeing patients with systemic sclerosis. I think you need to know when you’re tipped off to move the patient along to get some other expert’s help or opinion.
Healio: Should other specialties be brought in to co-manage these patients?
Pope: You can have your peers, where you might live near a center that has an expertise, and you might want to send the patient for research, co-management or to have them take over the patient if you’re not comfortable following. Also, pulmonologists and cardiologists are some that deal in pulmonary hypertension. Pulmonologists are the ones that deal with ILD, and there are also ILD subspecialists among the pulmonologists. Gastroenterologists are very helpful for us. Sometimes nephrologists if someone’s GFR is going down or they have a renal crisis. Rarely hematologists.
Healio: What are some first-line therapies that can be used in the presence of these complications?
Pope: Talking about ILD, first-line treatment — and this is also true for mild-to-moderate early diffuse patients with mild-to-moderate skin involvement and/or ILD — is often mycophenolate mofetil. After that, it could be switching to or adding rituximab (Rituxan, Genentech), which is 100% off-label. Tocilizumab (Actemra, Genentech), which isn’t usually reimbursed, and then for severe, progressive pulmonary fibrosis, in addition to immune suppression, you might be thinking about an anti-fibrotic drug.
For pulmonary arterial hypertension, No. 1, it needs to be proven by right-heart catheterization. The disease is treated according to mild, moderate or severe prognosis. For mild disease, there is treatment using monotherapy. For moderate or severe disease, you’re usually starting with two drugs, or, rarely, three drugs. It is treat-to-target. In both diseases, exercising the patient and treating hypoxia are important.
Healio: Do you have anything else to add?
Pope: When you get someone that you think is sick, please send them along to an expert center. Like anything with a rare disease, if you’re at an expert center, you can get more questions answered, but also have better outcomes. Not because the other doctors aren’t good, they’re very good, but because they are not experts in that area.
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