HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION
A 78-year-old woman, a 25-pack-year ex-smoker with no other known cardiovascular risk factors or previous personal or family cardiological history, who presented with a history of polyarthralgia and Raynaud's phenomenon that had not been studied, on chronic treatment with non-steroidal anti-inflammatory drugs.
He presented with symptoms of acute heart failure of three days' duration, with orthopnoea and oliguria, associated with oedematisation of the lower limbs. She denied chest pain or fever, but did recognise asthenia and loss of appetite in the last two months.
The blood pressure (BP) on arrival at the ED was 158/97 mmHg and heart rate (HR) 97 bpm, saturating at 92% with Ventimask at 50% FiO2. Afebrile. Physical examination revealed poor general condition with respiratory distress and jugular ingurgitation, and cardiopulmonary auscultation revealed no murmurs, with crackles up to mid-pulmonary fields. There was also oedema in the lower limbs up to the middle third of the pretibial region.
The presence of sclerodermic lesions on both hands and on the trunk is noteworthy.

COMPLEMENTARY TESTS
ANALYTICS: glucose 136 mg/dl; urea 130 mg/dl; creatinine 3.76 mg/dl; glomerular filtration rate 10 ml/min (previously normal); uric acid 6.2 mg/dl; alkaline phosphatase 86 U/l; GPT 32 U/l; NT-proBNP 2745 pg/ml; C-reactive protein 28.3 mg/dl; haemoglobin 10.5 g/dl; leucocytes 7.8 x 10000/mm3; platelets 117 x 10000/mm3; ESR 125 mm/h; prothrombin rate 86%; urine sediment with proteinuria; basal blood gas with pH 7.34, pO2 55 mmHg, pCO2 34 mmHg.
ELECTROCARDIOGRAM (ECG): sinus rhythm at 85 bpm with left bundle branch block.
THORAX RADIOGRAPHY: anteroposterior study with cardiomegaly and acute pulmonary oedema.
Transthoracic echocardiography: non-dilated left ventricle (LV) with mild concentric hypertrophy and prolonged diastolic relaxation pattern. LVEF 40%. Right ventricle (RV) slightly dilated with normal systolic function. Mild mitral insufficiency.
Mild tricuspid insufficiency that allows estimating a RV-AD gradient of 42 mmHg. Moderate pericardial effusion without involvement of the right chambers or transmitral flow variation.
HIGH RESOLUTION COMPUTATIONAL TOMOGRAPHY (HRCT): ground-glass images in both lower lobes in particular, suggesting pulmonary fibrosis.
Increased pulmonary artery calibre (35 mm) suggesting pulmonary hypertension.
CORONARYGRAPHY: epicardial arteries without significant angiographic lesions.

CLINICAL EVOLUTION
Diagnosed with acute pulmonary oedema and acute renal failure, he was initially admitted to the acute cardiac care unit, where respiratory support measures were established with non-invasive mechanical ventilation, with good adaptation and achieving clinical and gasometric improvement two hours after starting treatment. However, oligoanuria persisted despite the initiation of intravenous diuretic treatment, so renal replacement therapy with haemofiltration was finally chosen, which facilitated water discharge and contributed to the patient's clinical improvement.
Once the patient was initially stabilised, transthoracic echocardiography was performed, showing moderate left ventricular systolic dysfunction without frank asynergies.
With the presence of an unknown left bundle branch block (previous studies without intraventricular conduction disorders), a coronary angiographic study was performed, which showed no lesions in the epicardial bed.
The patient subsequently evolved favourably, being able to dispense with ventilatory support and starting haemodialysis sessions due to persistent oligoanuria. The presence of sclerodermic skin lesions, pericardial effusion, pulmonary hypertension on echocardiography and acute renal failure led to the suspicion of an autoimmune disease, so an immunological study was requested which showed positivity for antitopoisomerase I antibodies (AntiScl-70), and a pulmonary imaging study with CAT scan showed bibasal pulmonary infiltrates suggestive of pulmonary fibrosis with signs of pulmonary hypertension.
Treatment was started with glucocorticoids and angiotensin-converting enzyme inhibitors (ACE inhibitors) and continued with haemodialysis sessions, with echocardiographic monitoring showing only partial resolution of the pericardial effusion, with no other changes. Finally, in view of the favourable evolution, discharge home was decided.

DIAGNOSIS
Acute pulmonary oedema.
Moderate pericardial effusion.
Moderate left ventricular dysfunction.
Diffuse systemic sclerosis with scleroderma renal crisis.
Interstitial lung disease with fibrosis and pulmonary hypertension.
