One problem above the diaphragm and one problem below the diaphragm

Transcription

1 One problem above the diaphragm and one problem below the diaphragm Wouter Meersseman, MD, PhD Universitary Hospital Leuven General Internal Medicine Leuven 13 Dec 2013

2 83-year old lady Past medical history of breast carcinoma (6 years earlier) and ovarian cancer (24 years earlier); migraine First visit to ER: episode of central chest pain, two days duration, relief with nitrates - mild cough, no dyspnea Clinical examination: bilateral crackles, sat 100%, HR 130/min, irregular, no fever

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5 Lab results Variabele Normal values Hemoglobine 11,5 g/dl Hematocrit 34% White cell count 11, % neutrophils 83% % lymphocytes 7,5% % eosinophils 1,9% < 6 Creatinin level 0,64 mg/dl Albumin level 42 g/lit AST 22 U/lit < 32 LDH 349 U/lit C-reactief protein level 176 mg/lit < 5 Troponin T level < 0,02 microgram/lit < 0,13 D-dimer level 1076 microgram/lit < 500

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7 On the geriatric ward Developed fever (only one day, spiked up to 38,5 C) Started with amiodarone and amoxycillinclavulanate (presumed mild retrocardial pneumonia) Ultrasound heart CT scan of the chest

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10 Second admission one week later Increased dyspnea + chest dyscomfort Relapse palpitations Subfebrile up to 37.9 C Medication at home: citalopram, bisoprolol, low dose furosemide, marcoumar Clinical examination: mild signs of heart failure, decreased breath sounds, bilateral coarse crackles

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13 Despite multiple courses of antibiotics: persistence of CRP

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16 Nt probnp : 1500 Additional results ANA titre 1/80, subtyping negative panca titre 1/40, ELISA negative Protein electophoresis: mild polyclonal gammopathy (19 g/lit) Tuberculin skin testing: positive

17 Pleural fluid 4 times examined: culture negative, acid fast stain negative 4 times cytology: no malignant cells found Mild exudate - LDH 216 U/lit - glucose 106 mg/dl - total protein 38 g/lit - cells: 0,3 x 10 9 /lit, 38% segments

18 Pleural fluid analysis The glucose level of 106 mg/dl fairly excludes rheumatoid pleurisy, tuberculosis, empyema Protein content of 38 g/lit is compatible with exudate (caveat: patient is on diuretics) LDH level of 216 exludes empyema Low amount of white blood cells suggests absence of empyema, sarcoidosis, TBC

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20 Two months later No final diagnosis Went home and was readmitted, repeated tabs MRI of heart: mild signs of exudative pericarditis, no constrictive pericarditis Peristence of pleural fluid (PET-negative) + raised inflammatory markers No malignancy, no tbc Pleuroscopy (18/01/2011): no significant lesions

21 What would you do with this patient?

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23 30-year old lady Referred by general pratictioner to a gastro-enterologist Nausea, vomiting intermittently during the past two months and mild lower abdominal pain No relevant past medical history except for migraine Smokes 15 cigarettes a day Gastroscopy showed mild hemorrhagic gastritis, clinical examination??

24 Lab results Variabele Normal values Hemoglobine 9,5 g/dl Hematocrit 30% MCV fl White cell count 9, Platelets 472, , ,000 Urea 127 mg/dl < 30 Creatinin level 18,98 mg/dl Uric acid 9,8 mg/dl 4-7 sodium 134 mmol/lit potassium 7,3 mmol/lit 3,5-5,0 C-reactief protein level 47 mg/lit < 5 Sedimentation rate 120 mm after 1 hr < 30 D-dimer level 785 microgram/lit < 500

25 Clinical examination Not in distress Not very ill Temp 37.7 C, blood pressure 150/69 mmhg, pulse 72/min Euvolemic, no raised CVP Normal heart sounds Bilateral pain in the lateral abdominal region upon palpation

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32 Retroperitoneal fibrosis Inflammatory and fibrous retroperioneal tissue that often encases the ureters, the vessels or abdominal organs (Ormond s disease) Pathogenesis? Frequently idiopathic 1, exaggerated local inflammatory response around atherosclerotic aorta 2, as part of systemic auto-immune response

33 If not idiopathic, think off Malignancy (lymphoma, carcinoid tumors, sarcoma, colorectal, breast, prostate or bladder cancer) Infectious (tuberculosis, histoplasmosis, actinomycosis) Radiation therapy Retroperitoneal haemorrhage Drugs (bromocryptin, methyldopa, ergotderivatives, hydralazine) IgG4 related disease, lupus, Erdheim- Chester

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35 Maybe we shouldn t idiopathic in our case

36 Symptoms retroperitoneal fibrosis

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38 Do both patients share similarities?

39 Do they share similarities? They both have inflammation They both have non resolving fluid or tissue accumulation

40 83-year old lady 30-year old lady

41 Pathogenesis Well known for valve abnormalities Induction of 5HT3 receptors (serotonin) Mitogenesis Similar to carcinoid lesions - cabergoline, pergolide (PERMAX) - dihydro-ergotamine, ergotamine, methysergide (DIHYDERGOT, DYSTONAL) - Fenfluramin, Ecstasy

42 Final diagnosis: Cafergot-induced - pleural and pericardial effusion resolved by colchicine - retroperitoneal fibrosis, resolved by steroids