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CASE

CASE. Esen KIYAN, MD Istanbul University Faculty of Medicine Department of Respiratory Disease. 24 y, female, housewife (married and has one child). Complaint: Dyspnea and cough Exertional dyspnea for five years (progressive increase in dyspnea)

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CASE

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  1. CASE Esen KIYAN, MD Istanbul University Faculty of Medicine Department of Respiratory Disease

  2. 24 y, female, housewife (married and has one child) • Complaint:Dyspnea and cough • Exertional dyspnea for five years (progressive increase in dyspnea) • Painful red-purple spots on the hand and foot finger tips, night sweat, and intermittent fever for the last 1.5 year • Fatigue • Investigated for these complaints (1.5 year ago) • ESR: 90mm/hour, CRP 41mg/L • Hb 10g/dL, Hct %35 • ANA and RF (-), c and p-ANCA (-)

  3. Past history: • Jaundice at age 13 • Abortion due to intrauterine growth retartation Family history: nonspecific

  4. Investigated at Internal Medicine and Rheumatology clinics and specific diagnosis (-). No further investigatione because of pregnancy. • Complaints continued after the delivery(dyspnea, painful spots at the finger tips of hand and foot, intermittent fever,fatigue). • For the last five months dry cough and palpitation, for the last three months weight loss (10kg)Admission to an outpatient clinic

  5. Chest-X-Ray

  6. ENT examination: Normal • High ESR 18 Eylül 2007 90mm/hour 26 Eylül 2007 110mm/hour • Anemia (Hb 9g/dL, Hct %30) • Urine examination:Normal • Thyroid function tests: Normal

  7. C3-C4: normal Anti-Jo1 Anti-RNP Anti-Scl70 Anti-Sm Anti-SS-A(Ro) Anti-SS-B(La) p ve c-ANCA RF ve ANA Anti dsDNA Anti CCP Anticardiolipin (IgG ve M) NEGATIVE NEGATIVE

  8. For fever of unknown etiology • Wright (-) • Gruber Widal (-) • Abdominal US: Normal

  9. During outpatient evaluation • Flantadin tb • LABA+ICS • LTA • Geralgine K • Antibiotics tried

  10. Admission to a chest clinic • Physical examination: • painful red spots on hand finger tips, • Respiratory system findings normal

  11. ESR:62 mm/saat CRP:39 mg/L Leucocyte:5000 /µl (%61 PNL, %31 Lymp) Hb: 9.8 g/dL Hct: %30.4 PLT: 246000/µl MCV:80.1 µm3 MCH: 25.9 pg MCHC: 32.3 g/dL BUN: 13 mg/dl Creatinin: 0.9 mg/dl Sodium: 140 mmol/L Potassium: 4 mg/dl Calcium: 8,9 mg/dl Glucose: 67mg/dl LDH: 122 U/L ALP: 260 U/L AST:12 U/L ALT:9 U/L GGT: 12 U/L T.Protein:6.7g/dl Albumin:4.4 g/dl LABORATORY FINDINGS ECG:sinusal tachycardia

  12. Lung Function Tests • FVC 2050 ml (%61) • FEV1 2040 ml (%70) • FEV1/FVC %100 • MEF25-75 3820 ml (%95) DLCO: %70, room air SaO2 SaO2%96

  13. Whatis your diagnosis? • Vasculitis • Connective tissue diseases • ILD/Sarcoidosis • Pulmonary hemosiderosis • Other

  14. Lung CT

  15. Which investigation do you choose ? • Echocardiography • Bronchoscopy • Open lung biopsy • Biopsy from skin lesions • Perfusion lung scanning

  16. D-Dimer:8.80µg FEU/ml (N <0.5) • Perfusion lung scanning: Irregular perfusion at both lung bases (low probability for PE)

  17. BRONCHOSCOPY • EBL (-), mucosa hyperemic and fragile, and increased capillarity Mucosa biopsy: - Subepithelial neovascularization and edema Bronchial lavage - smear negative for ARB (-), culture (-) - Nonspecific cultures and cytology (-)

  18. Patient hospitalized (Chronic dyspnea and cough etiology?) • Anemia, painful spots at the finger tips of hands • BP:100/70mmHg, Pulse:110/dk, Respiratory rate: 18, SaO2%96 • Respiratory system: no pathologic finding • Loud S1, 2/6 middiastolic murmur at mitral area, 3/6 murmur at mesocardiac area

  19. ECHO:Left atrial mass, 2(+) MR, 3(+) TR, Severe PHT (PAP 90mmHg)

  20. POSTOPERATIVE DIAGNOSIS: MYXOMA • Beningn primary cardiac tumor (%80 located in the left atrium) • Dyspnea,palpitation, syncope. Of cases, 30% has fever and weight loss • Lab: Elevated ESR and CRP, leucocytosis and anemia • Heart failure and systemic emboli

  21. Myxoma and Lung • Pulmonary edema • Pulmonary HT • Pulmonary emboli • Right heart failure

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