Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival

Abstract

Antisynthetase syndrome is characterised by the association of interstitial lung disease and myositis with different anti-tRNA-synthetase antibodies. The occurrence, aetiology and prognosis of pulmonary hypertension have not yet been evaluated.Among 203 consecutive patients, transthoracic echocardiogram and right heart catheterisation results were retrospectively analysed in the light of clinico-biological, morphological and functional parameters. Definitions of pulmonary hypertension were based on the European Society of Cardiology/European Respiratory Society 2009 guidelines, with severe pulmonary hypertension being defined by a mean pulmonary arterial pressure .35 mmHg.Pulmonary hypertension was suspected by transthoracic echocardiogram in 47 (23.2%) cases, corresponding to pulmonary hypertension ''possible'' (n527, 13.3%) or ''likely'' (n520, 9.9%). Right heart catheterisation was performed in 21 patients, excluding pulmonary hypertension in five and confirming pre-capillary pulmonary hypertension in 16 (7.9%). Although related to interstitial lung disease in all cases, pre-capillary pulmonary hypertension was severe in 13 (81.3%) patients (mean¡SD pulmonary arterial pressure 46¡9 mmHg), frequently associated with low cardiac index (mean¡SD 2.3¡0.8 L?min -1 ?m -2 ) and high forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio (2.5¡0.6). Pulmonary hypertension was significantly associated with a lower survival rate (p,0.001), with a 3-year survival rate of 58%.The occurrence of pulmonary hypertension in antisynthetase syndrome is significant and dramatically worsens the prognosis. Although systematically associated with interstitial lung disease, pulmonary hypertension was usually severe, suggesting a specific pulmonary vascular involvement. @ERSpublications PH in antisynthetase syndrome significantly worsens the prognosis, suggesting a specific pulmonary vascular involvement

Interstitial lung disease-associated antisynthetase syndrome

“…Physiotherapy can be beneficial in improving deconditioned muscles, but has no benefit in reversing fibrosis. [8,9] Patients are able to do more with improved physical strength and with a greater understanding of the condition and how to manage it. [5,8,9] This is evident from the improvement in the distance covered and dyspnoea rating during the 6-minute walk test.…”

“…[5,8,9] The timely and appropriate treatment with NIV prevented the deterioration during the acute setting. [1,6,8,9] Flow (L/s ) 1 2 We suggest that in the appropriate clinical context, patients with multisystem involvement presenting with arthralgias, Raynaud's phenomenon, myositis and lung involvement should be assessed for the ASS. A negative test for antinuclear antibodies (ANA) does not exclude the ASS syndrome.…”

Pulmonary hypertension: Classification and pathobiology

“…Pulmonary hypertension is a comorbidity that can be found in all connective tissue diseases, including sys temic sclerosis, systemic lupus erythematosus, poly myo sitis/dermatomyositis (antisynthetase syndrome) [19] and rheumatoid arthritis. Epidemiological studies suggest that pulmonary hypertension is a common finding in patients with systemic sclerosis (more than 10% of patients [20]).…”

Pictorial review: Thoracic involvement in connective tissue diseases: radiological patterns and follow-up

“…A ways associated with ILD. PAH is usually severe, associated with a lower survival rate, suggesting the specific pulmonary vascular involvement (30)(31)(32)(33)(34).…”