Personal history
Allergic to penicillin. No known hypertension or diabetes mellitus.
Dyslipidaemia under dietary treatment. Denies toxic habits. Primary autoimmune hypothyroidism in substitutive treatment since 2011. Euthycocic delivery of singleton gestation 40+1 weeks on 15/06/2014 without immediate complications. Hyperemesis gravidarum. Baseline situation: active and independent. Adequate for her age.
Surgical interventions: removal of cutaneous nevus on 4 occasions, one of which was dysplastic.
Chronic treatment: levothyroxine 150 mcg (75 mcg after delivery): 1 tablet/day. Iron proteinsuccinylate 40 mg: 1 vial per day. Present illness A 30-year-old woman attended the emergency department on 21/06/2014 due to poor general condition with thermometric fever of 38.5°C accompanied by urinary discomfort with dysuria, pollakiuria and pain in the right lumbar region for 24 hours. He presented with nausea but no vomiting. No cough or expectoration or other accompanying symptoms. The patient was discharged on 17/06 after euthecological delivery without immediate complications.

Physical examination
Blood pressure: 110/90 mmHg. Heart rate: 90 beats/minute. Temperature: 38.8 °C. Oxygen saturation 100%. Weight 63.5 kg, height 1.72m. BMI: 21.46 kg/m2.
Cardiac auscultation: rhythmic sounds at 90 bpm and no murmurs.
Pulmonary auscultation: preserved vesicular murmur. Abdomen: soft and depressible, painful on palpation in the hypogastrium and fist, positive right renal percussion.
Lower extremities: oedema with fovea up to the knee with no signs of thrombosis.
Gynaecological examination: engorged breasts with no signs of infection. Vagina ample and elastic. Non malodorous discharge.

COMPLEMENTARY TESTS
Admission blood tests: glucose 81, creatinine 0.99, sodium (Na+) 139, potassium (K+) 4.2, CRP 11.39, haemoglobin 10.6, haematocrit 32.4%, leucocytes 13380 (neutrophils 77%), platelets 275,000. INR 0.93. Abnormal and sediment: pH 7, haemoglobin 593 red cells/field. Leukocytes 1912 leukocytes/field. Bacteria 2019/uL. Pyuria.
Other analytical findings on admission: basal ACTH 1180, basal cortisol 3.3. TSH 8.08, T4 1.16, T3 1.8. Plasma Na+ 124, urine Na+ 15, plasma chlorine 89, urine chlorine 10, urine osmolarity 294, blood osmolarity 288. Anti-striated muscle/myocardial antibodies: negative. Anti-adrenal antibodies: high positive.
Chest X-ray: congestive hilarity. Enlargement of the broconvascular tract. Bilateral costophrenic sinus impingement with volume decrease in right hemithorax.
ECG: sinus tachycardia at 100 bpm. PR 0.12sec. Narrow QRS with indeterminate axis and BIRDHH morphology. Negative T waves in II, III and aVF. Deep T waves in precordial leads.
Transvaginal ultrasound: involuted uterus with collapsed cavity. No impression of debris.
Abdominal ultrasound: moderate amount of free intraperitoneal fluid, predominantly in the flanks and right iliac fossa, and to a lesser extent in the lower pelvis. Kidneys of normal size and echostructure, without dilatation of the excretory system. Severe right pleural effusion with almost total pulmonary atelectasis and moderate left pleural effusion.
Transthoracic echocardiography (on the Cardiology ward): biventricular dysfunction without dilatation of cavities (LVEF 35%). Mild mitral insufficiency. Signs of low cardiac output. Absence of indirect signs of severe pulmonary hypertension.
Coronary angiography: coronary arteries without significant lesions.
Ventriculography: dilated left ventricle with irregular contour on the underside (small aneurysms). LVEF 25%. Moderate MI. TDVI 24 mmHg. Severe ventricular dysfunction.
Endomyocardial biopsy: fragment of myocardium with mild hypertrophic features without inflammation or granulomas. No interstitial fibrosis is seen. No iron or glycogen deposition. No inflammation suggestive of myocarditis.
Cardiac MRI: no dilatation of cavities. Myocardial thickness normal Severe left ventricular dysfunction (LVEF 35%). No intramyocardial oedema. Negative late enhancement, making the diagnosis of myocarditis and ischaemic heart disease highly unlikely.  Microbiology: blood culture: negative after 6 days of incubation.
Urine culture: Escherichia coli >100,000 CFU/ml sensitive to gentamicin, fosfomycin, cotrimoxazole and amoxicillin-clavulanic acid.
Parvovirus B19 serology: Ac IgG and Ac IgM parvovirus B19 negative.

EVOLUTION
The patient was admitted to gynaecology with a diagnosis of acute pyelonephritis in a pregnant woman. Urine and blood cultures were requested and intravenous treatment was started with gentamicin 80 mg every 8 hours, clindamycin 900 mg every 8 hours, serotherapy (1500 cc per day) and analgesia. On 23/06 the patient presented nausea, vomiting with dizziness and hypotension (BP 97/73 mmHg) and 500 cc of extra serum therapy and metoclopramide were administered. The following morning the patient persisted with general malaise and dyspnoea despite 100% saturation, which was attributed to an anxiety crisis. However, in the following hours the patient presented poor general condition with mucocutaneous pallor, sweating and peripheral coldness as well as tachycardia and tachypnoea. Examination revealed: HR 120 bpm, RR 40 rpm, BP 80/30mmg, O2 Sat 88% Pulmonary auscultation: marked bibasal hypoventilation in the right lung base. Urgent chest X-ray was performed, showing bilateral pleural effusion; urgent abdominal ultrasound, showing free intra-abdominal fluid and bilateral pleural effusion. Laboratory tests showed Na+ 128, K+ 6.3, NT-proBNP 9162, Hb 9.2, D-dimer 8421, CRP 37.42, procalcitonin 4.50, GOT 1190, GPT 377, TSH 7,690, T4 0.96, pH 7.20, pCO2 19, HCO3 12, lactate 4 and glucose 40. Given the haemodynamic deterioration of the patient with respiratory claudication, she was admitted to the ICU with a diagnosis of metabolic acidosis secondary to sepsis of urinary origin due to E. coli.
The patient was admitted to the ICU on the afternoon of 24/06, very unstable with sustained hypotension and signs of tissue hypoperfusion requiring initial respiratory support with non-invasive mechanical ventilation and administration of 3 doses of hydrocortisone due to acute pulmonary oedema. An ECG was performed showing negative T waves on the inferior and precordial sides, as well as elevated markers of cardiac damage with peak CKMB 21.6 and troponin T 279 and NT-proBNP 12626. An urgent echocardiogram was performed showing severe systolic dysfunction with global hypokinesia, dilatation of the chambers with LVEF 10% and a cardiac output of 2 litres/minute was calculated. Given that the patient was in cardiogenic shock, treatment with vasoactive drugs with dobutamine and noradrenaline was started, which allowed systolic blood pressure to be maintained at 80 mmHg and cardiac output to improve to 5 litres/minute and LVEF 35%. With suspicion of myocarditis/ischemic cardiomyopathy, cardiac catheterisation with ventriculography and endomyocardial biopsy, cardiac MRI, parvovirus B19 serology and cardiac immunology were all negative, so the patient was attributed to possible peripartum cardiomyopathy complicated by sepsis of urinary origin. Treatment was started with bromocriptine, continued with gentamicin and associated trimethoprim-sulfamethoxazole. The patient improved clinically, with resolution of the bilateral pleural effusion, improvement in the infectious parameters and cardiac output, allowing the progressive withdrawal of vasoactive drugs and on 01/07 she was transferred to the Cardiology ward.  Initially on the ward the evolution was favourable, allowing beta-blockers and ACE inhibitors to be started at low doses. The control echocardiography showed improvement in systolic dysfunction with LVEF 35%. However, on the night of 03/07 the patient presented nausea and dizziness with hypotension of 70/40mmHg. A blood test was performed with the following alterations: creatinine 1.65, urea 58, Na+ 117, K+ 6.4, which were confirmed. Bromocriptine and trimethoprim-sulfamethoxazole were suspended (antibiotic cycle had been completed) in case the alterations were secondary to drugs, and corrective measures for hyperkalaemia and serum therapy were administered. Given that the patient at 24-48 hours persisted with symptoms of dizziness, nausea and hypotension and with peak analytical alterations on 06/07 with creatinine 1.99, urea 90, Na+ 115, K+ 7.4, ACTH and basal cortisol were requested and IV hydrocortisone was administered on suspicion of acute adrenal insufficiency. The patient progressively improved with treatment. Endocrinology was consulted and the patient was classified as having acute adrenal insufficiency in a patient with intense stress due to urinary sepsis and cardiogenic shock due to peripartum cardiomyopathy. The patient received steroid treatment in the ICU, so the condition had remained masked. Given the haemodynamic stability of the patient and the resolution of the ionic alterations, she was discharged from hospital on 08/07/2014.  Treatment at discharge: omeprazole 20 mg 1c/24h, ramipril 2.5 mg 1c/24h, bisoprolol 2.5 mg 1c/24h, furosemide 40 mg 1/2 c/24h, levothyroxine 75 mcg 1c/24h, hydrocortisone 20 mg 1c/8h.

Evolution at discharge: at 4 months follow-up echocardiography was performed, showing improvement with LVEF 52%. At the endocrinological level, the analytical results confirmed the condition as acute adrenal insufficiency of autoimmune origin. After 9 months, the patient was asymptomatic and was living an active and independent life.

DIAGNOSIS
Urinary sepsis due to Escherichia coli
Cardiogenic shock due to peripartum cardiomyopathy with severe systolic dysfunction
Acute adrenal insufficiency-addisonian crisis
