CASE

Esen KIYAN, MD

Istanbul University Faculty of Medicine

Department of Respiratory Disease

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 (-)

24 y, female, housewife (married and has one child)

Past history:

Jaundice at age 13

Abortion due to intrauterine growth retartation

Family history: nonspecific

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

Chest-X-Ray

ENT examination: Normal

High ESR 18 Eylül 2007 90mm/hour26 Eylül 2007 110mm/hour

Anemia (Hb 9g/dL, Hct %30)

Urine examination:Normal Thyroid function tests: Normal

C3-C4: normalAnti-Jo1Anti-RNPAnti-Scl70Anti-SmAnti-SS-A(Ro)Anti-SS-B(La)p ve c-ANCARF ve ANA

NEGATIVE

Anti dsDNA

Anti CCP

Anticardiolipin

(IgG ve M)

NEGATIVE

For fever of unknown etiology

Wright (-)Gruber Widal (-)Abdominal US: Normal

During outpatient evaluation

Flantadin tb LABA+ICS LTA Geralgine K Antibiotics tried

Admission to a chest clinic

Physical examination: painful red spots on hand finger tips, Respiratory system findings normal

LABORATORY FINDINGS

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

ECG:sinusal tachycardia

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

What is your diagnosis?

1. Vasculitis

2. Connective tissue diseases

3. ILD/Sarcoidosis

4. Pulmonary hemosiderosis

5. Other

Lung CT

Which investigation do you choose ?

1. Echocardiography

2. Bronchoscopy

3. Open lung biopsy

4. Biopsy from skin lesions

5. Perfusion lung scanning

D-Dimer:8.80µg FEU/ml (N <0.5)

Perfusion lung scanning: Irregular perfusion at both lung bases

(low probability for PE)

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 (-)

BRONCHOSCOPY

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

Patient hospitalized (Chronic dyspnea and cough etiology?)

ECHO:Left atrial mass, 2(+) MR, 3(+) TR,

Severe PHT (PAP 90mmHg)

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

Myxoma and Lung

Pulmonary edema Pulmonary HT Pulmonary emboli Right heart failure