FRI0299 Pulmonary Arterial Hypertension in Idiopathic Inflammatory Myopathies: Data from The French Pulmonary Hypertension Registry and Review of The Literature

Abstract

BackgroundOccurrence of pulmonary arterial hypertension (PAH) in idiopathic inflammatory myopathies (IIM) without extensive interstitial lung disease (ILD) has been rarely described.ObjectivesThis study aimed to report all cases with association of PAH and IIM in the French Pulmonary Hypertension (PH) Registry, to identify IIM features associated with PAH, and to describe treatment modalities of these patients.MethodsAll cases of IIM-PAH were retrieved from the French PH Registry, which gathers PAH patients prospectively enrolled by 21 referral centers across France. Patients were excluded if they had an extensive ILD or overlap syndrome. Characteristics of IIM-PAH patients were compared to a control group of IIM patients without PH.ResultsAmong the 5223 PAH patients in the Registry, 34 had a diagnosis of IIM. Among them, 3 IIM-PAH patients had no sign of extensive ILD or overlap syndrome, and were included in this study. In these patients, dermatomyositis (DM) was the only identified IIM. Two of them had a myopathic DM, confirmed by electromyography and biopsy. The last patient had an amyopathic DM, associated with anti-Ku antibodies. Pre-capillary PH was hemodynamically proven in all patients. PAH always developed after IIM onset, was severe in all cases, and led to a marked functional impairment.By pooling our cases with 6 previously reported patients, and comparing them to a control cohort of 35 IIM patients without PH, we identify several IIM characteristics possibly associated with PAH occurrence, including DM subtype (78% vs. 46%, p=0.02), skin involvement (p=0.04), anti-SSA antibodies (p=0.05) and peripheral microangiopathy (p=0.06).Overall, IIM-PAH patients were managed by corticosteroids and/or immunosuppressants, either alone or combined with PAH therapy. Patients that did not receive PAH therapy did not seem to respond to IIM treatment alone.ConclusionsOur study reports for the first time the rare but possible association of PAH and IIM in a large prospective PH Registry. In that setting, PAH seems associated with DM, skin involvement, peripheral microangiopathy and anti-SSA positivity. The best therapeutic strategy for IIM-PAH remains to be defined.Disclosure of InterestNone declared

Pulmonary arterial hypertension in connective tissue disorders: Pathophysiology and treatment

“…The authors concluded that the best therapeutic strategies are yet to be defined in pSS-PAH. Finally, considering the nine cases reported by Sanges et al 24 in the French PAH registry, IIM-PAH does not appear to respond to glucocorticoids and/or immunosuppressants alone, whereas PAH-specific therapy appeared to be effective in stabilizing the disease.…”

Lung Diseases in Inflammatory Myopathies

“…102 A recently described series of nine patients identified DM subtype, skin involvement, and presence of anti-SSA autoantibodies as features more particularly associated with pulmonary hypertension in subjects with IIM. 102 Treatment of pulmonary hypertension using phosphodiesterase-5 inhibitors (sildenafil, tadalafil), endothelin receptor antagonists (bosentan, ambrisentan), and/or prostacyclin derivatives (intravenous epoprostenol, subcutaneous treprostinil, nebulized iloprost) should be reserved for the rare patients with PAH (i.e., without associated significant ILD), as group 3 pulmonary hypertension is believed to represent a complication of chronic hypoxia. 103 Distinguishing between group 1 PAH and group 3 pulmonary hypertension may be difficult, however, in subjects with ILD of mild to moderate severity.…”

Lung Involvements in Rheumatic Diseases: Update on the Epidemiology, Pathogenesis, Clinical Features, and Treatment

“…In the majority of cases, myositis-associated lung involvement takes a form of ILD. Unlike SSc, PAH is often secondary to ILD and isolated PAH is rarely found [ 151 ]. Although not a primary lung involvement of myositis, aspiration pneumonia and hypoventilatory respiratory failure are two serious complications related to pharyngeal and respiratory muscle involvement, respectively.…”