Background and aims Scleroderma is a rare disease that can involve any internal organ. Systemic scleroderma is usually associated with restrictive cardiomyopathy.

Methods We report the case of a patient diagnosed as having systemic scleroderma since 1975, with cutaneous, pulmonary, digestive involvement, referred to our department for dyspnea on mild exertion, productive cough (mucous sputum), and anterior thoracic pain not related to exertion. All symptoms started about 4 months prior to referral and were very little influenced by corticotherapy and 2 courses of antibiotics administered prior to admission in our department.Clinical examintaion reveals telangiectasia over the cheek bones, microstoma, spontaneous amputations of distal phalanges fingers 2–4 right hand, basal bilateral pleural friction rub, apical systolic murmur grade 3.

Results Blood examination shows leukocytosis, polyglobulia, dyslipidemia normal ESR and CRP.ECG: sinus rhythm, HR=96/min, QRS axis at +10, LVH, negative T wave (anterior territory). Respiratory functional evaluation shows obstructive dysfunction (reduction by 30% of FEV1). Cardiac ultrasound reveals interventricular septum of 18 mm thickness, posterior wall of left ventricle of 15 mm thickness, with an intraventricular pressure gradient of 60 mmHg at rest.

Conclusions Our patient has the severe, obstructive form of cardiomyopathy. This was the cause of the respiratory symptoms, despite the interstitial pulmonary disease. As a consequence, we changed diltiazem to verapamil, reduced the vasodilators and the corticosteroid doses, which lead to the net improvement of the symptoms. In systemic scleroderma, respiratory symptoms must not be taken “for granted” as being produced by interstitial lung disease., but must be evaluated correctly and completely at each consult.