Eight Parameters Recommended to Monitor for PH in JSSc Patients

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by Maureen Newman |

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When young patients develop juvenile systemic sclerosis (JSSc), it is vital to monitor them for changes in cardiopulmonary parameters that may lead to conditions such as pulmonary hypertension. However, there are no current guidelines that describe which cardiopulmonary parameters are most vital to monitor in order to screen for diseases and begin necessary treatments. Dr. Ivan Foeldvari, as part of an international, collaborative research effort, spearheaded this concern by developing recommended screening parameters for monitoring patients with JSSc and presenting his findings at the 2014 American College of Rheumatology Meeting.

shutterstock_210735535Dr. Foeldvari’s presentation, entitled “Cardiopulmonary Involvement in Juvenile Systemic Sclerosis: Development of Recommendations for Screening and Investigation,” described a number of parameters that were chosen as most helpful and relevant in following a JSSc patient’s disease progression. The parameters were derived from a meeting of members from the Pediatric Rheumatology European Society (PRES) Scleroderma Working Group and pediatric cardiologists. Agreement among members was defined as 75% consensus.

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As a result of the discussions, eight baseline cardiopulmonary measurements should be obtained for patients newly diagnosed with JSSc: blood pressure, 12 lead electrocardiogram (ECG), 24 hours ECG, echocardiogram (ECHO) with Doppler, cardiac magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT) of the thorax, pulmonary function test (PFT), and six-minute walk test (6MWT). Afterwards, monitoring for changes should be continued for at least five years following diagnosis.

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During these five follow-up years, 12 lead ECG, ECHO with Doppler, 6MWT, and PFT should be repeated every six months; 24 hour ECG should be repeated annually; and HRCT should be repeated once at the three-year mark. Of course, if other signs of disease are present, such as chest pain upon exertion, tests may need to be conducted more often and other tests such as right heart catheterisation should be conducted if pulmonary hypertension is suspected.

Although these recommendations were derived from only one group of experts, the consensus opinions provide excellent starting grounds for a monitoring plan tailored to JSSc patients. According to Dr. Foeldvari, “The objective of an aggressive screening program is to identify cardiopulmonary and gastrointestinal involvement at a stage which may be amenable to treatment. The recommendations developed by this group aim to standardize care and improve outcomes in this rare disease.”

A Conversation With Rare Disease Advocates