HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION

History
Cardiovascular risk factors: Smoker since youth. Currently about 6 cigarettes/day (cumulative consumption 20 packs/year) Heavy drinker until about 3 years ago. Currently occasional drinker. Former cocaine user (last use 9 years ago). Arterial hypertension for 5 years and without treatment, with good control. Obesity grade II-III in the past, currently overweight. Cardiological history: no personal or family history of early ischaemic heart disease or sudden death. Other history: cutaneous psoriasis since childhood, without arthropathy. One episode of erythroderma 15 years ago. Chronic treatment: occasional treatment for psoriasis: clobetasol propionate ointment and calcipotriol cream.

Present illness
The patient comes to the emergency department for progressive dyspnoea until minimal effort of seven days of evolution. Orthopnoea and paroxysmal dyspnoea the previous night. He also reported rapid palpitations with a frequency measured at home of around 130 beats per minute. No angina pectoris, no episodes of dizziness or syncope. No other relevant symptoms in the anamnesis by apparatus, except for the presence of skin lesions related to psoriasis and skin striae as the only relevant complaint.

During his stay in the emergency department and despite initial treatment with intravenous furosemide and beta-blockers, the clinical situation worsened and he was transferred to the coronary unit.

Physical examination
Afebrile, heart rate 135 bpm, blood pressure 141/99 mmHg. RR 22 rpm and basal oxygen saturation 94% without supplementary O2 on arrival (evolution in the first hours: RR 32/min. SpO2 with O2 at 4 l/min 92-94%). General appearance: erythroderma with multiple reddish plaques with scaling (suggestive of psoriasis) on the trunk and upper extremities. Stretch marks on abdomen, arms and legs. Cold hands and feet, with thinned fingers and toes and signs of chronic malperfusion. Rhythmic carotid arteries, no murmurs. Cardiac auscultation: rhythmic, tachycardic, systolic murmur in mitral focus II/VI with preserved second sound. Pulmonary auscultation: bibasal crackles. Abdomen: soft, depressible, non-painful, no palpable masses or megaliths. Lower limbs: symmetrical pedal pulses, pretibial oedema, signs of chronic venous insufficiency with varicose veins. Diffuse psoriasis lesions, with reddish plaques with scaling and inflammatory signs on the trunk and extremities.

COMPLEMENTARY TESTS
Chest X-ray: cardiomegaly with vascular redistribution, Kerley's B lines and bilateral alveolar infiltrate. Electrocardiogram on admission: uncommon atrial flutter with 3:1 conduction. Narrow QRS with normal axis, QS in V1-V3, QR in V4, ST elevation of 2 mm in V4-V5, qR in inferior leads, compatible with old anterior and inferior infarction. Normal QTc interval. Complete blood test: Glucose 123 mg/dl, sodium 137 mmol/l, potassium 4.1 mmol/l. Creatinine 0.66 (FGE 120 ml/min). US Troponin T: 780 ng/l- 738 ng/l - 686 ng/l (normal < 14 ng/l). Liver function tests: GGT 144 U/l (LSN: 30 U/l), FA 139 U/l (LSN: 117 U/l), LDH 634 (LSN: 480 U/l). Transaminases normal. TSH: 0.86 mIU/l. Lipid profile: total cholesterol 194 mg/dl, LDL 133 mg/dl, HDL 36 mg/dl, triglycerides 127 mg/dl. Anaemia profile: iron 35 ug/dl, ferritin 434 ng/ml, IST 13.2%. Rheumatoid factor 10.8 kU/l (LSN: 15 kU/l). Interleukin 6: 19 pg/ml - 35 pg/ml -16 pg/ml (normal < 6). Proteinogram: acute inflammatory pattern. Immunoglobulins: IgA 5.19 g/l (0.7-4), C4 0.45 g/l (0.1-0.4). Rest normal. Autoimmunity: ANA positive (titre 1/80, homogeneous pattern). ANA negative. ANCA negative. Negative antiphospholipid antibodies. Cryoglobulins: negative. Kappa and Lambda free light chains: normal levels. HIV, HBV and HCV serology: negative. Quantiferon TB: positive 1.26 UI/ml.

Transthoracic echocardiography: left ventricle of normal thickness, slightly dilated. LVEF severely affected (28%) at the expense of severe hypokinesia of the mid anterior and lateral wall segments and all apical segments. Relaxation pattern not assessable by wave fusion. Right ventricle of normal size and function. Moderately dilated left atrium (area 33 cm2). Aortic valve trivalve, normofunctioning. Mitral valve with slightly thickened leaflets, with central regurgitation jet of moderate insufficiency (ERO 0.36 cm2, MR volume 35 ml, intermediate density Doppler). Mild tricuspid insufficiency. PSAP of 48 mmHg + PVC (normal). The presence of intraventricular thrombus is ruled out with contrast.
Diagnostic coronary angiography: Severe coronary artery disease of three vessels: middle and distal anterior descending artery, first diagonal branch, distal circumflex artery, distal right coronary artery and posterolatereal branch. Very bad distal beds, diffusely diseased. No response to intracoronary nitroglycerin. Beds not revascularisable. Computed tomography angiography: thoracic aorta of normal dimensions. Supra-aortic trunks without alterations. Patent abdominal aorta. Celiac trunk, common hepatic artery and splenic artery of fine calibre with a perivascular cuff. The splenic artery is very thin distally, showing hypoperfusion in the spleen at this stage. Superior and inferior mesenteric arteries of fine calibre with perivascular cuff. Common iliac arteries, external iliac arteries, common femoral and superficial femoral arteries with decreased calibre and perivascular cuffing. Conclusion: findings suggestive of medium vessel vasculitis. Positron emission tomography: No increase in glucose metabolism in the wall of the large arterial vessels suggestive of vasculitis is visualised. It should be remembered that corticosteroid treatment causes false negatives and that the sensitivity of PET is lower in medium and small vessel vasculitis. Two hypermetabolic foci were identified in the colon, one in the descending colon (SUVmax 8.09) and the other in the sigma (SUVmax 9.13), which we recommend evaluating by colonoscopy to rule out malignant tumour pathology. Doppler ultrasound of supra-aortic trunks: Left carotid axis with a small plaque at the level of the bulb, predominantly fibrolipid, with a smooth and regular surface, which does not cause haemodynamic alterations. Normal flows in left CCA, ICA and CEA. Right carotid axis with normal echostructure. Vertebral arteries permeable and orthodromic. Abdominal X-ray: dilated large bowel loops with an image suggestive of cecal volvulus in the right hypochondrium.

Computed tomography of the abdomen: cecal volvulus, probably partial, of the cecal scale type. Mural endoluminal polyps in the splenic angle of the colon, measuring 15 mm, and in the rectosigmoid junction, measuring 11 mm, both with uptake in the previous PET-CT study. Radiological improvement of the signs of vasculitis of the celiac trunk and superior and inferior mesenteric arteries. A more prominent perivascular cuff persists in the primitive and external iliac vessels with probably significant stenosis of femoral arteries. Colonoscopy: the ileum is reached. In the cecum there are aphthae arranged in a linear fashion, covered with fibrin compatible with ischaemic colitis. Two polyps were observed: one of them measuring about 15 mm in the descending colon. No other lesions were observed. Biopsy of skin, subcutaneous cellular tissue, muscle and sural nerve: non-specific inflammatory findings with no evidence of vasculitis. Electroneurography/electromyography: patchy involvement of motor and sensory fibres of the right ulnar, peroneal and tibial nerves suggesting mononeuritis multiplex. Echocardiography at discharge: severely dilated left ventricle (indexed VTD 158 ml/m2). Akinesia with thinning of all apical segments. Medial anterior akinesia and medial anterior, medial anterolateral and medial inferolateral septum. Medial inferior hypokinesia and basal inferior septum. Severe systolic dysfunction (biplane Simpson calculation with Sonovue LVEF 25%). No apical thrombus after echocontrast injection. Restrictive pattern diastolic dysfunction with elevated filling pressures. Severe left atrial dilatation. Mild right atrial dilatation. Normal sized right ventricle with preserved function. Severe functional mitral regurgitation. Thin leaflets with symmetrical tenting of the leaflets. Central regurgitant jet with dense, wide, holosystolic flow with severe regurgitation. ERO 0.6 cm2. Mild aortic insufficiency. Mild tricuspid insufficiency. Pulmonary hypertension at rest.

CLINICAL EVOLUTION
In the coronary unit, amiodarone perfusion and oxygen therapy with high-flow glasses was started, achieving clinical stabilisation in the first 24 hours, with reversion of flutter to sinus rhythm at 90 bpm, negative balances of almost 3 litres and marked clinical improvement. The first echocardiography and coronary angiography were performed. In view of the catheterisation findings, an assessment was requested from the internal medicine department with the suspicion of a vasculitic condition as the cause of the coronary lesions. That same day, treatment was started with corticosteroid boluses, oral prednisone and later methotrexate as an immunosuppressant. From the clinical point of view, the patient was admitted for a very long time, with a hospital stay of almost 40 days. Cardiologically, the patient showed a rapid and favourable evolution during the first week and was discharged to the conventional hospital ward. However, on the seventh day of admission (after the PET scan), he presented an arrhythmic storm with three episodes of sustained ventricular tachycardia that required electrical cardioversion and multiple non-sustained episodes. For this reason, he had to be transferred again to the coronary unit and was started on lidocaine perfusion, midazolam and beta-blocker with a good response. He did not suffer any more arrhythmic events and presented a slow but adequate evolution of his heart failure. During the second week, the patient suffered a paralytic ileus, with suspicion of associated cecal volvulus (which was the reason for requesting an abdominal CT scan and colonoscopy) which improved with digestive rest, nasogastric tube and antibiotic treatment without the need for surgery. Lastly, it should be mentioned that on admission the patient had very severe skin involvement, with erythroderma secondary to his psoriasis, as well as involvement of the hands and feet with signs of poor perfusion that slowly improved with medical treatment. As for the aetiological diagnosis, computed tomography angiography showed lesions suspicious of vasculitis in the visceral arteries and in both iliac arteries without involvement of the supra-aortic trunks. The complete blood test showed a rather anodyne result with elevation of several non-specific inflammatory markers (CRP, ferritin, complement, IL-6...) but with negative results in the key tests (ENA, ANCA, cryoglobulins, antiphospholipid antibodies, HBV, HCV and HIV serology...). In view of the results, PET-CT was ordered in the hope of confirming inflammation data at the vascular level to support the presumptive diagnosis (at that time Takayasu-type large vessel vasculitis was the main candidate). However, the absence of uptake in the supra-aortic trunks ruled out this pathology. Following this setback, multiple diagnostic studies were requested with the intention of confirming medium-vessel vasculitis (mainly polyarteritis nodosa, PAN). A skin, subcutaneous tissue and nerve biopsy was performed, as well as an electromyogram/electroneurogram. It is worth mentioning that the initial intention was to perform a direct arterial biopsy of the iliac/femoral territory (a territory known to be affected by the scan data). However, this option was discarded following vascular surgery assessment which considered, given the calibre of the vessels involved, that the risk of complications (ischaemia and/or haemorrhage) was too high. The biopsy found no data suggestive of vasculitis, but the electromyogram showed a pattern of mononeuritis multiplex, very typical of PAN. The clinical course was favourable and he was discharged after implantation of an automatic defibrillator (ICD) for secondary prevention. As for the diagnosis, it was concluded (given the vascular involvement, the multiple mononeuritis and the improvement with anti-inflammatory drugs) that the most likely cause of the patient's clinical picture was polyarteritis nodosa. However, given the multiple cardiovascular risk factors (including cocaine use), early atherosclerosis was always considered as a possible alternative. She was discharged with immunosuppressive and steroid treatment, oral anticoagulation, acetylsalicylic acid, statins and full medical treatment of heart failure, to continue outpatient follow-up in the advanced heart failure unit.

DIAGNOSIS
PAN type vasculitis with coronary, celiac, mesenteric and femoral involvement.
Ischaemic dilated cardiomyopathy with severe left ventricular dysfunction. Heart failure in acute pulmonary oedema on admission, euvolemic on discharge. Non-revascularisable three-vessel coronary artery disease (diffuse arterial disease compatible with vasculitis).
Arrhythmic storm. ICD implantation in secondary prevention.
Uncommon atrial flutter with rapid ventricular response, pharmacological cardioversion to sinus rhythm.
Ischaemic colitis.
