Case Studies Highlight Diversity of Rheumatoid Arthritis-Associated PH

Case Studies Highlight Diversity of Rheumatoid Arthritis-Associated PH

A recent article that presented case studies of three patients with rheumatoid arthritis-associated pulmonary hypertension (PH), highlights the diversity of the underlying causes of the disease, and emphasizes the importance of diagnosing it early.

The study “Rheumatoid arthritis associated pulmonary hypertension: Clinical challenges reflecting the diversity of pathophysiology” was published in the journal Respiratory Medicine Case Reports.

Rheumatoid arthritis (RA) can contribute to multiple manifestations of chronic lung disease, including PH. The prevalence of PH in RA is infrequent, but has been estimated to be between 0.8% to 21-27.5% based on echocardiographic data. Now, the authors presented three cases to emphasize the importance of a thorough evaluation and accurate diagnosis.

In the first case, a 60-year-old woman with a known history of RA and co-existing pulmonary fibrosis with chronic respiratory failure, presented with life-threatening low oxygen levels. She was transferred urgently to the respiratory intensive care unit (RICU). Due to the co-existing lung fibrosis, this was a difficult case to diagnose and treat.

Testing involved electrocardiograms, blood test, heart echocardiograms, pulmonary function tests, chest CT, and finally right heart catheterization. These tests confirmed PH and the patient was treated with the combination therapy Ventavis (iloprost) and Revatio (sildenafil).

She was discharged 15 days after oxygenation levels improved. At the one-year follow-up evaluation she was stable under oxygen therapy.

This case, the authors wrote, highlights the importance of early identification of PH as the patient received her diagnosis inside the RICU during severe respiratory failure.

Although PH usually occurs in the context of interstitial lung disease, the second case is an example of isolated PH in the absence of severe interstitial disease, classified as pulmonary arterial hypertension.

A 68-year-old male patient with RA presented with shortness of breath on exertion and respiratory failure. Previous treatments with immunosuppressive drugs were unable to control his arthritis. An array of tests involving chest X-rays, lung function tests, chest CT, echocardiograms, and right heart catheterization suggested PH.

The patient started treatment with oxygen, Volibris (ambrisentan), and the diuretics Lasix (furosemide) and Aldactone (spironolactone). This treatment combination initially improved the patient’s symptoms and exercise capacity.

However, after seven years, the patient complained about more severe shortness of breath on exertion. This deterioration in lung capacity was attributed to the progression of vasculopathy (a condition affecting blood vessels). New therapy with Adcirca (tadalafil) and Ventavis (iloprost) were added to the treatment regimen, and symptoms improved.

Although immunosuppressants are beneficial in controlling lung disease in other connective tissue disorders, the effect of these drugs for RA on the evolution of PH appears controversial in the current literature, and more studies are needed the authors noted.

Finally, the third case was more complex because the patient had several significant c0-variables contributing to the development of PH.

An 80-year old woman with a smoking history, RA, left heart diastolic dysfunction, and abnormal curvature of the spine (kyphoscoliosis) presented with shortness of breath on exertion, and severe functional limitations. She also had a previous pulmonary embolism that was treated with oxygen and the anticoagulant Sinthrome (acenocoumarol).

After a ventilation perfusion scan, a diagnosis of chronic thromboembolic disease was confirmed, and right heart catheterization with pulmonary angiography confirmed PH. The patient started treatment with Adempas (riociguat), and her symptoms were improved.

These three cases emphasize the importance of early identification of PH in patients with RA. Because  multiple treatment options are available, symptoms can be treated and progression to more serious complications can be avoided.

“Rheumatoid arthritis associated pulmonary hypertension can be attributed to interstitial lung disease, vascular disease, and chronic thromboembolic disease. Chronic inflammation reduces patient’s functional capacity and conceals early symptoms of cardiovascular disease and pulmonary hypertension.” the team concluded. “RHC [right heart catheterization] is the gold standard method for the diagnosis of PH. Early diagnosis and treatment prevents right heart failure and significantly enhances quality of life.”

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4 comments

  1. Joanne Sperando says:

    My brother was diagnosed with RA a few years before he was diagnosed with PH. Our PH is genetic. Our maternal aunt passed away from PH in 1963, my brother was diagnosed at 34 years old and passed 20 years after in 2015. I was diagnosed 3 years after my brother and have been battling PH for 18 years. Our PH doctor always said that PH and RA were linked in my brother’s case. I hope the researchers continue to figure this out.

    • Tim Bossie says:

      We are sorry to hear about your brother and the diagnosis of PH. It is truly heartbreaking to see people struggle with one chronic illness and then develop another that is more severe. We hope that as research continues in this field that we not only learn more, but are able to better treat it… or even stop it.

  2. Kathy L. says:

    I recently went to a major hospital affiliated pulmonary institute, where I thought I would get a solid diagnosis of RA/PH, but a clear diagnosis eluded the junior physician who evaluated me. He ultimately missed the mark, I am and always have been a seronegative, so unfortunately he ended up contributing all of
    my symptoms to a cardiac condition, which ultimately was disproved after findings of a PET CT showed a previously large (9mm) spiculated lung nodule now resolved within just a matter of weeks, and most likely due to an inflammatory condition. A lot of patients (seronegative) are being overlooked or outright misdiagnosed because of a failure to see that patient in a “different light” when compared to a seropositive. My quality of life is poor at this point, and it didn’t have to be this way.

    • Tim Bossie says:

      Hi Kathy. Thank you for sharing your difficult experience with us and our readers. You are right in that there are still many misdiagnosis. This can be because of many different factors, but ultimately boils down to awareness and education.

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