HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION

The patient is a 57-year-old male, a former smoker, dyslipidaemic and with a history of chronic ischaemic heart disease. One year ago, after suffering unstable angina with positive ergometry, coronary angiography was performed which revealed coronary artery disease of the anterior descending artery, which was revascularised by implanting a drug-eluting stent. He also had gastro-oesophageal reflux and had undergone surgery for an inguinal hernia. He was currently being treated with aspirin, torasemide and pantoprazole.
He attended the emergency department of our centre for progressive dyspnoea of 4 months' evolution associated with intense asthenia, generalised weakness, weight loss of 25 kg in this period and frequent episodes of diarrhoea. There had been a clear clinical worsening of the dyspnoea in the last two weeks to the point of minimal effort, together with the appearance of oedema in the lower limbs. He denied episodes of central chest pain, orthopnoea or paroxysmal nocturnal dyspnoea. He also reported no cough, expectoration, fever or night sweats or bleeding.
On arrival at the ED, blood pressure (BP) was 110/60, heart rate (HR) was 90 bpm and baseline O2 SatO2 was 96%. Physical examination revealed increased jugular venous pressure, a flail chest and the presence of Raynaud's phenomenon in both hands. Cardiopulmonary auscultation revealed rhythmic heart sounds with a systolic murmur in the mitral focus and crackles in the left base. He also had oedema with fovea up to the knee.

COMPLEMENTARY TESTS
Baseline electrocardiogram: RS at 75 bpm, PR 200 ms, right bundle branch block (RBBB) with signs of right ventricular (RV) overload.
Chest X-ray: cardiothoracic index (CTI) at the upper limit of normality, possible paratracheal lymphadenopathy. Minimal vascular redistribution.
Laboratory tests:
Biochemistry: glucose 89, creatinine 0.75, urea 36, uric acid 8.5, Na 140, K 4, NTproBNP 1800, CRP 50. Interferon B negative, B2-microglobulin 3.27, ANA 1/640 with rest of autoimmunity study normal, complement normal, tumour markers negative. Serology negative.
Haemogram: Hb 9.4, leukocytes 6,400 (normal formula), platelets 66,000.
Coagulation: INR 1.4, PT 67%.
Echocardiogram: left ventricle of normal size and preserved function with mild hypertrophy (interventricular septum 12 mm). Diastolic dysfunction with pseudonormal mitral filling pattern (E/e' lateral side of 15). Right ventricle of normal size and function. No evidence of pulmonary hypertension at rest.
Mild mitral insufficiency without other associated valvulopathies. Mild pericardial effusion without echocardiographic data of haemodynamic compromise.
Cardiac MRI: moderate bilateral pleural effusion with passive atelectasis of the left lower lobe. Circumferential pericardial effusion with a maximum thickness of 2 cm on the right lateral aspect. Myocardial thickness preserved. Perfusion study without alterations. No pathological gadolinium deposits were observed. Biventricular function preserved.
Angio-CT of pulmonary arteries: no signs of pulmonary thromboembolism.
CT scan: small moderate right pleural effusion and left pleural effusion sheet. Both lung bases show a cobblestone pattern with bronchiectasis, parenchymal distortion and honeycomb cysts. Bilateral hilar adenopathy. Cardiomegaly and pericardial effusion. In conclusion: study compatible with non-specific interstitial pulmonary fibrosis that may correspond to collagen disease (systemic sclerosis).

CLINICAL EVOLUTION
The patient was initially admitted to our department for heart failure with associated pericardial effusion, and diuretic treatment was started with marked clinical improvement.
Once stable and having ruled out clinical and echocardiographic signs of cardiac tamponade and the presence of pulmonary thromboembolism by means of an angio-CT scan of the pulmonary arteries, a referral was made to the gastrointestinal department to study a diarrhoeal process secondary to possible inflammatory bowel disease and to internal medicine for a study of the general syndrome.
As part of the battery of tests performed during admission, a colonoscopy was requested in order to rule out digestive tumour pathology causing this general syndrome. After administering the preparation for this test, the patient suffered an episode of sudden dizziness and the electrocardiogram showed the presence of complete atrioventricular block with wide QRS leakage at 40 bpm, at which time he was being treated with low-dose atenolol. The patient was transferred to the coronary unit where a temporary electrode was implanted via the right jugular vein. After a negative chronotropic medication washout period and verification of the absence of adequate atrioventricular conduction, a DDDR bicameral permanent pacemaker was implanted without incident. He had a good postoperative course and was discharged.
Given the suspicion of an infiltrative cardiomyopathy secondary to a systemic disease with involvement of several organs, the patient was assessed by the rheumatology department and the internal medicine department. The study was completed with a CAT scan that showed a pattern in both bases suggestive of interstitial pulmonary fibrosis, a spirometry that showed moderate restriction and a slight decrease in diffusion and an electromyogram in which data compatible with myopathy were observed. In addition, a skin biopsy was performed which revealed histological findings compatible with scleroderma. With these data, and with the persistence of episodes of self-limited diarrhoea due to a probable malabsorptive component, the diagnosis of diffuse systemic sclerosis with cutaneous, cardiac (atrioventricular block, pericardial effusion), pulmonary (interstitial pulmonary fibrosis) and digestive (malabsorption) involvement was confirmed. Treatment with cyclophosphamide was started, with poor tolerance due to renal and bone marrow toxicity. Subsequently, he presented poor clinical evolution with progressive worsening of his functional class and suffered several admissions for heart failure with a predominance of congestion data refractory to diuretic treatment, and the patient died one year after diagnosis.

DIAGNOSIS
Diffuse systemic sclerosis with cutaneous, pulmonary, cardiac and digestive involvement.
Congestive heart failure with preserved EF.
Mild-moderate pleuro-pericardial effusion.
Complete atrioventricular block.
Implantation of DDDR bicameral definitive pacemaker.
Thrombopenia.
