Dilated-cardiomyopathy: unusual early manifestation of crest syndrome

Author: 
Garvit Garg., Hetal Pandya ., Singh V.P., Sejal Sejwani and Jaydutt Patel

Systemic Sclerosis (SSc) which is also known as scleroderma is a multisystem autoimmune disease characterised by vasculopathy, organ impairment, immune abnormalitiesand dermal fibrosis. SSc incidence is about 2.7-19.3 new cases per million adults per yearadults with a female to male predominance of 4.6: 1. The presence of cardiac involvement in SSc is often seen in 20% to 25% of patients which is associated with a 70% mortality at 5 years.
A 34 years old female presented with complain ofepisodic bluish discolouration of digits of upper limb and swelling over hands since 1 year. She has dyspnea on exertion since 2 months which was associated with palpitations since 1 month. She was tachypanic with tachycardia & was normotensive and had 99 % saturation on room air. Her face was mask like with loss of wrinkles and expressions with small mouth aperture. On CVS examination, S1 and S2 were heard with S3 and loud P2 with basal crackles. 2D echo was showing of all cardiac chambers were dilated with ejection fraction of 25%. Her ANA was positive with Anti-Scl70 antibody. Hence, the patient was diagnosis as DCMP (Dilated cardiomyopathy) in CREST syndrome. So, we are reporting this case because of development of cardiovascular complication (DCMP) in very early causes of systemic sclerosis.

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DOI: http://dx.doi.org/10.24327/ijcar.2017.5145.0660