Notes

I have a patient presenting with syncope. On workup found to have pulm HTN . Had Hyperextensible joints and we were called in for ANA pos STATus . Could you suggest any ideas if there is any rheumatologic association w/ joint hypermobility and pulm HTn

Did not have any sclerodactytly and physical exam was totally normal except for Hyperextensible jts. Her sister also had similar jts ,also had extra jts 3 jts in each finger in addition to MCP

sending studies below :



CT Angio: 04/01/2016 Pericardial effusion.

CXR 04/09/2016 The cardiac silhouette is enlarged, but stable. Small patchy opacities in the right lower lung may be due to central vascular congestion versus developing infiltrate. Calcified adenopathy within left hilum. Scattered calcified granulomas are noted in the lungs. No pneumothorax or pleural effusion is seen. The bones are unchanged.
Convexity in AP window may represent a prominent pulmonary artery.

ECHO:
Systolic septal flattening consistent with RV pressure overload. Left ventricular systolic function is normal.Ejection Fraction = 55-60%. Grade I diastolic dysfunction, (abnormal relaxation pattern). The right ventricle is moderate to severely dilated. The right ventricular systolic function is mildly reduced. There is severetricuspid regurgitation with Severe Pulmonary Hypertension. Small, predominantly posteriorly located,pericardial effusion, with no echocardiographic indications of cardiac tamponade


anti Ro/La -negative,
ESR negative,
ANA positive, RF 690, anti CCP 227.9.
SCL 70 positive