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Pulmonary Arterial Hypertension in Connective Tissue Diseases – A Devastating Complication
intravenous diuretics. When patients are stabilised with this treatment,
Case of Pulmonary Arterial Hypertension Associated with
conversion to subcutaneous treprostinil is proposed, since this treatment is
Systemic Sclerosis
also feasible for patients with hand problems such as digital ulcers, sclerosis
of the fingers and arthritis. Although in theory the application of a
subcutaneous system in patients in whom the disease affects the skin could A female patient born in 1950 was referred to the pulmonary
cause dosage problems or more severe side effects, such as pain at the hypertension clinic for analysis of dyspnoea on exertion in June
infusion site, this treatment is found to be effective and well-tolerated in 2004. She had had complaints of progressive dyspnoea during the
the vast majority of CTD patients at our centre. previous eight months, with occasional near syncope. Furthermore,
she had suffered from a Raynaud’s phenomenon in the last five
Immunosuppressive Therapy months. At physical and laboratory examination her blood pressure
To date, no data are available to support the use of immunosuppression was normal, but scleroderma signs were found, such as decreased
in PAH associated with SSc. However, a few case reports and a mouth opening, teleangiectasia, subtle sclerodactyly and ANA
retrospective study suggested that a minority of patients with PAH positivity. Pulmonary function testing revealed a decreased DLCO
associated to SLE or MCTD could benefit from immunosuppression using (34% of predicted), hypoxemia at rest and a 6MWT distance of
cyclophosphamide intravenous pulse therapy and glucocorticosteroids.
34
197m with desaturation. Echocardiography revealed signs of
In our practice, immunosuppression is applied in patients when other pulmonary hypertension, with an estimated PAP of 55/25mmHg. A
signs of active CTD are present, and patients are always treated with high-resolution computed tomography (HRCT) scan of the chest
specific PAH medication when their functional class is NYHA III or IV. An showed oesophageal dilatation, but was otherwise unremarkable,
example of the treatment scheme is shown in the box opposite. as were the other tests used (as described in Figure 2). At right heart
catheterisation, the PAP was 67/45mmHg and wedge pressure was
Summary 8mmHg. Her exercise capacity was classified as NYHA III. Besides
In summary, the treatment of patients with PAH associated to CTD can oxygen therapy, treatment with bosentan was initiated: her exercise
differ from the treatment of idiopathic PAH. In general, this complication is capacity improved to NYHA class II and her 6MWT distance
more aggressive and, despite the slow rise of more or less regular screening improved to 420m. From September 2006 onwards, her 6MWT
schemes, patients tend to present with more severe PAH and their life distance decreased slightly to 400m, and in December 2006 it was
expectancy is worse. The specific treatments for PAH are found to be further reduced to 360m and she was reclassified as NYHA III.
effective in CTD-related-PAH patients, although long-term results are Sildenafil 20mg trice daily was added, without effect. Subcutaneous
lacking. With this in mind, as well the fact that doctors already know their trepostinil was initiated and a dosage of 20ng/kg/min was achieved
at-risk population, screening schemes consisting of an annual after two weeks. After 12 weeks she was once again NYHA class II,
echocardiography combined with a thorough history and pulmonary with her 6MWT distance improved to 403m.
function testing, and easily accessible right heart catheterisation in cases of
doubt, provide us with an unique opportunity to start timely treatment. ■
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EUROPEAN MUSCULOSKELETAL REVIEW 2007 47
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