SESSION TYPE: Cardiovascular Student/Resident Case Report Posters II
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: There have been cases previously reported that bring out a temporal relation between Pulmonary Arterial Hypertension (PH) and Multiple Sclerosis (MS). These case reports implied a possible causal relationship between the Interferon (IF) β therapy in MS and PH. We present two cases with MS and PH
CASE PRESENTATION: The first patient is a 41 yr old female with idiopathic PH diagnosed in 2007 who presented with NYHA class 3 dyspnea. A right heart catheterization (RHC)was performed which revealed mean Pulmonary artery pressure (MPAP) of 39 mm Hg , Pulmonary capillary wedge pressure (PCWP) of 7 mmHg. Secondary causes of PH were ruled out. She was started on Sildenafil and Epoprostenol for the treatment of PH with marked clinical improvement. The patient subsequently developed paraesthesias in her feet and numbness in her face which was thoroughly evaluated and was diagnosed with MS. She has had several attacks and relapses of MS requiring steroid bursts. She is currently maintained on Glatiramer for her relapsing remitting MS. The second patient is s 63 year old female with MS since her 3rd decade of life maintained on Glatiramer for several years. At the age of 59, she developed NYHA class 2 dyspnea .RHC was performed which showed a MPAP of 29 mm Hg and a mean PCWP of 15mmHg. A focused workup for causes of PH was performed, which was essentially negative. Chest X-ray and pulmonary function test indicated no evidence of lung parenchymal disease. At this point, a diagnosis of idiopathic PH was made. She was started on Sildenafil and Flolan with improvement in her dyspnea but progressive worsening of MS with development of neurogenic bladder.
DISCUSSION: Gorbett et al through their case series proposed a cytokine mediated mechanism for vascular changes resulting in the development of PH in patients with MS treated with IF β. As both our patients were never treated with IF β, it is unlikely that the development of PH was secondary to use of IF β and hence the above hypothesis does not explain the association of MS and PH in them. This provokes the idea if an autoimmune mediated demyelination in MS has a likeness to pulmonary vasculature changes associated with the development of PH.
CONCLUSIONS: The aim of this case series is to highlight that there may be more than a mere coexistence of the two conditions. There probably is an independent association between PH and MS which needs further investigation. We also emphasize the prudence of early screening, diagnosis and initiation of treatment of PH in these patients to decrease the morbidity associated with it in an already debilitating condition like MS.
1) Ledinek AH, Jazbec SS, Drinovec I, Rot U; Pulmonary arterial hypertension associated with interferon beta treatment for multiple sclerosis: a case report.Mult Scler. 2009
DISCLOSURE: The following authors have nothing to disclose: Pooja Swamy, Ghulam Saydain, Kalyan Kosuri
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