CASE PRESENTATION

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CASE PRESENTATION
Prof. Dr. Birsen MUTLU I.U. Cerrahpasa Medical
Faculty Department of Pulmonology
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H. B.

• 56 years-old
• Female
• Born in Trabzon, lives in Istanbul
• Housewife
• Married

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COMPLAINTS

• Dyspnea
• Dry cough
• Weakness
• Swelling legs
• Dry mouth
• Dry eyes

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HISTORY

• Experiencing dyspnea for ten years on effort
  (climbing up stairs)
• For the last one year, complaining about dyspnea
  and weakness even during her daily activities
• The patient, who started to complain about
  dyspnea while resting 2-3 months ago and the
  legs of whom started to swell, was hospitalized
  for further examination and treatment.

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• HISTORY Hypertension (15 years)
• Diabetes mellitus Type 2 (5 yrs)
• FAMILIAL HISTORY Nothing special
• Addiction None
• Allergy Undefined

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• REGULARLY USED MEDICINE
• Trimetazidine Hcl (Vastarel ) 20 mg tb 3x1
• Ramipril (Delix ) 2,5 mg tb 1x1
• Rosiglitazone maleate (Avandia ) 4 mg tb 1x1
• Glimepride (Glirid ) 1 mg tb 2x1

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PHYSICAL EXAM - 1

• Conscious, cooperative
• Dyspnea, orthopnea ()
• Body temp37 ºC
• Obese
• Cyanosis (-)
• Palpable lymphadenomegaly (-)
• Clubbing (-)
• Raynauds phenomenon (-)

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PHYSICAL EXAM - 2

• RESPIRATORY SYSTEM
• Respiration per minute 20
• Two hemithoraces equally contribute to
  respiration
• Respiratory sounds decreased bilaterally, in
  both lower zones, Late inspiratory crackles heard

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PHYSICAL EXAM - 3

• CARDIO - VASCULAR SYSTEM
• Apex beat 80/mins, rhythmic
• TA 110/70 mmHg
• Heart sounds S1-S2 (), grade 2/6 systolic
  murmur at tricuspid and pulmonary area
• ABDOMEN
• No organomegali
• No painful region
• No mass and fluid

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PRE - DIAGNOSIS ???

1. Pulmonary Hypertension
2. Left Heart Failure
3. Interstitial Lung Disease
4. CTELD
5. Cor Pulmonale

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CHEST X-RAY
H.B. 04. 06. 2007
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PRE - DIAGNOSIS ???

1. Pulmonary Hypertension
2. Left Heart Failure
3. Interstitial Lung Disease
4. CTELD
5. Cor Pulmonale

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LABORATORY FINDINGS
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HAEMOGRAM

• WBC 9200/mm³
• Hb 12,2 gr/dL
• Hct 36,5
• PLT 259000/mm³

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• Sedimentation 38 mm/h
• CRP 21,5 mg/L
• BCG Scar 2 scars
• PPD 11 mm enduration
• Blood Biochemistry Normal
• Comp Urine Count Normal

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WHAT WOULD YOU FIRST PROPOSE FOR DIAGNOSIS ???

1. Computerized Tomography
2. Lung Function Tests
3. TTD Ecocardiography
4. ECG
5. Serological Examination for Vasculitis

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COMPUTERIZED TOMOGRAPHY
k
l
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COMPUTERIZED TOMOGRAPHY
rv
ra
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COMPUTERIZED TOMOGRAPHY
rv
ra
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COMPUTERIZED TOMOGRAPHY
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HRCT
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HRCT
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ECG
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TT DOPPLER ECHOCARDIOGRAPHY

• Right cardiac spaces are extended
• Left ventricle EF 60
• Moderate tricucpid insufficiency
• Pulmonary arterial peak systolic pressure 85
  mmHg

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RIGHT HEARTH CATHETERIZATION

• PULMONARY ARTERIAL PRESSURE
• Before adenosin 75/38 mmHg,
• mean 50 mmHg
• After adenosin 60/38 mmHg,
• mean 45
  mmHg

Vasoreactivity negative
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PRE - DIAGNOSIS ???

1. Pulmonary Hypertension
2. Left Heart Failure
3. Interstitial Lung Disease
4. CTELD
5. Cor Pulmonale

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SPIROMETRE
28
SPIROMETRE
29
LUNG VOLUMES
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DIFFUSION CAPACITY
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ARTERIAL BLOOD GASES

• PO2 7O,8 mmHg
• PCO2 40,5 mmHg
• SaO2 94,2
• PH 7,46
• HCO3 28,9 mmol/L
• ? Room condution

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PULMONARY HYPERTENSION

• Hemodynamic definition (Right Heart
  Cateterization)
• Average of PAP gt 25 mmHg while resting
• Average of PAP gt 30 mmHg in exercise
• Pulmonary wedge pressure lt 15 mmHg

ACCP Evidence-based clinical practice guidelines
Chest 2004 WHO, 2003 Galie N, Torbicki A, Barst
R, et al.Guidelines on diagnosis and treatment of
pulmonary arterial hypertension. Eur Heart J
2004 25 2243-2278
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CLASSIFICATION OF PULMONARY HYPERTENSION(Simonnea
u et al. J Am Coll Cardiol 2004)
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6DYT FUNCTIONAL CLASS

• 6DYT
• Distance 240 m
• SaO2 93 - 87
• Borg scala dyspnea 3-9
• NYHA IV

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ROMATOLOGICAL MARKERS

• p ANCA
• c ANCA
• MPO ANCA
• PR3 ANCA
• Anti SSA
• Anti SSB
• Anti SCL 70
• ANTI JO1

• RF 48,6 (0-15)
• ANA slightly brindled ()
• Anti ds DNA
• Anti SM
• Anti SM/RNP
• C3C, C4

negative
negative
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SCHIRMERS TEST

• Right eye 20 mm
• Left eye 17 mm
• Normal

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WHAT WOULD YOU FIRST PROPOSE FOR DIAGNOSIS ???

• A. BAL
• B. Transbronchial Biopsy
• C. Salivary Gland Biopsy
• D. Open Lung Biopsy
• E. Transthoracic Biopsy

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LUNG BIOPSY

• Lung biopsy is not recommended as a routine due
  to the risks involved
• It might be exceptionally performed with the
  condition that a specific problem is answered
  through tissue diagnosis

Quality of evidence Experts view Benefit Good
Suggestion power E/A
Badesch DB, Abman SH, Simonneau G, Rubin LJ,
McLaughlin VV. Medical Therapy for Pulmonary
Arterial Hypertension. Updated ACCP
Evidence-Based Clinical Practice Guidelines.
Chest 2007 13119171928
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WHAT WOULD YOU FIRST PROPOSE FOR DIAGNOSIS ???

• A. BAL
• B. Transbronchial Biopsy
• C. Salivary Gland Biopsy
• D. Open Lung Biopsy
• E. Transthoracic Biopsy

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DIAGNOSIS
SJÖGRENS SYNDROME
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SJÖGRENS SYNDROME

• It is a slowly-proceeding, chronic, systemic and
  auto-immune disease that is characterized by the
  lymphocitic infiltration of exocrine glands.
• It proceeds mainly by dryness in the mouth and
  eyes ? Sicca syndrome
• Prevalance 0,5-5 (mostly asymptomatic)
• ?/? 9/1
• Age 40-60

Kelleys textbook of rheumatology. Philadelphia
Saunders, 20051105-24
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SJÖGRENS SYNDROME

• 50-60 is composed of secondary Sjögrens
  syndrome

• Rheumatoid arthritis
• Systemic lupus erythematous (SLE)
• Scleroderma
• Mixed connective-tissue disease
• Primary biliary cirrhosis
• Myositis
• Thyroiditis
• Vasculitis
• Chronic active hepatitis
• Mixed cryoglobulinemia

Clinical Manifestations and Early Diagnosis of
Sjögren Syndrome Arch Intern Med.
20041641275-1284
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CLINICAL FINDINGS

• Exocrine gland disease
• Nonexocrine organ disease
• 
  
• Arthralgia/Arthritis 60
  
  
• Raynauds phenomenon 37
  
  
• Lymphadenomegaly 14
  
  
• Lung involvement 14
  
  
• Vasculitis
  11
  
• Kidney involvement 9
  
  
• Liver involvement 6
  
  
• Lymhoma 6
  
  
• Splenomegaly 3
  
  
• Peripheral neuropathy 2
  
  
• Myositis
  1
  

? Radiological involvement
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Harrisons principles of internal medicine. 16th
ed. 2005. 1990-1993
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LUNG INVOLVEMENT

• Interstitial lung disease
• Nonspesific interstitial pneumonia
• Lymphocitic interstitial pneumonia
• Bronchiolitis obliterans organized pneumonia
• Usual interstitial pneumonia
• Desiccation of tracheobronchial tree
• Diffuse or focal nodular infiltrates
  (pseudolymphoma, malignant lymphoma, amiloidosis
• Pulmonary vasculitis and primary pulmonary
  hypertension (rare)
• 
  

• Interstitial lung diseases
• Marvin I. Schwarz Talmadge E. King Mosby year
  Book 2003568-572

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CRITERIA FOR DIAGNOSIS

• Symptoms for dryness in eyes
• Findings for dryness in eyes (Schirmers test
  5 mm/min)
• Symptoms for dryness in mouth
• Findings for dryness in mouth (unstimulated
  saliva secretion 1,5ml/15min
• 
  Parotid sialography , Scintigraphy )
• Minor salivary gland biopsy
• Autoantibodies (Anti-Ro / SS-A, Anti-La / SS-B)

4 criteria should be positive (one biopsy, one
autoantibody)
Classification criteria for Sjogren's syndrome a
revised version of the European criteria proposed
by the
American-European Consensus Group.Ann Rheum Dis.
200261(6)554-8.
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CLINICAL OBSERVATION

• Quensyl tb (Hidroxyclorkin) (2x1)
• Deltacortil tb (2x1)
• In the second month, the patient stopped quensyl
  by her decision

In the fourth month, she applied again due to
increase in her complaints
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CLINICAL OBSERVATION

• Dyspnea has become more severe
• Coughing has increased
• Bruises in hands and feet in the last 20 days
• Medical exam
• Dyspnea, orthopnea ()
• Cyanosis ()
• Pretibial edema /
• Respiratory sounds decrased in both lungs,
  prolonged expirium , wheezing ()
• Heart sounds 2/6 systolic murmur () at
  tricuspid and pulmonary area

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CHEST X-RAY
H.B. 11.01.2008
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PA GRAFILER
11.01.2008
04.06.2007
54
SPIROMETRY
04.06.2007
11.01.2008
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DIFFUSION CAPACITY
04.06.2007
11.01.2008

• DLCO 9,04 (48) mL/mmHg/min
• DLCO/VA 3,35 (67) mL/mmHg/min/L

• 9,02 (47)
• 4,97 (99)

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ARTERIAL BLOOD GASES
04.06.2007
11.01.2008

• PO2 7O,8 mmHg
• PCO2 40,5 mmHg
• SaO2 94,2
• PH 7,46
• HCO3 28,9 mmol/L

• PO2 48 mmHg
• PCO2 46 mmHg
• SaO2 84,8
• PH 7,45
• HCO3 30 mmol/L

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WHAT WOULD YOU THINK AT THIS POINT ???

1. The patient should be followed up
2. Immunsuppresive dose should be increased
3. Treatment for PH should be started
4. Another factor should be looked for
5. The disease is progressing

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2. COMPUTERIZED TOMOGRAPHY
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2. COMPUTERIZED TOMOGRAPHY
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2. HRCT
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WHAT WOULD YOU THINK AT THIS POINT ???

1. The patient should be continued to be followed
2. Immunsuppresive dose should be increased
3. Treatment for PH should be started
4. Another factor should be looked for
5. The disease is in progress

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2. TT DOPPLER ECHOCARDIOGRAPHY

• Right cardiac spaces are extended
• Left ventricle EF 60
• Slight tricuspid failure
• Pulmonary artery peak systolic pressure
  75mmHg
• (6
  months ago 85 mmHg)

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2. RIGHT HEART CATHETERISATION

• Pulmonary arterial pressure 77/35 mmHg
  (average 50)
• Atrial septal defect with shift from left to
  right!!!
• Transesophageal Echocardiogram proposed

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TRANSESOPHAGEAL ECOCARDIOGRAPHY

• An atrial septal defect with a 2,4 cm diameter
  was found in the interatrial septum
• It was discussed in the council of cardiology
  Cardiovascular surgery
• It was decided to make an operation to close ASD

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ATRIAL SEPTAL DEFECT (ASD)

• FREQUENCY
• Congenital heart disease is seen in 0,5-1,2 of
  infants
• ASD composes 7-11 of all congenital heart
  diseases and 30 of congenital heart diseases in
  adults
• It is the most frequently found new shant lesion
  in adolescence
• It can emerge at any age

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
67
ATRIAL SEPTAL DEFECT (ASD)

• SIZE
• Small 2 cm
• Large gt2 cm
• (Diameter is usually 2-4 cm)

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
68
ATRIAL SEPTAL DEFECT (ASD)

• TYPES
• Ostium Secundum (most frequent) ? 65- 75 is
  women
• Ostium primum
• Sinus venosus
• Coronary sinus defect

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
69
ATRIAL SEPTAL DEFECT (ASD)

• While secundum type adolescents and young adults
  are asymptomatic, more than half of them develop
  dyspnea, rapid fatigue, atrial flutter or
  fibrilation, right heart failure or pulmonary
  hypertension with the age of 40

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
70
ATRIAL SEPTAL DEFECT (ASD)

• In 5-10 of untreated ASD especially women
  a pulmonary vascular disease develops
• Their 10-year survival rate is found to be 95
  after suregery and 84 with medical treatment

Wu JC, Child
JS, Comoon congenital heart disorders in adults.

Curr Probl Cardiol
2004. 29641-700 Webb G, Gatzoulis MA Atrial
septal defects in the adultRecent progres and
overview

Circularin 20061141645-1653
71
PH TYPES WHEN DIAGNOSED
Portal hypertension

• 2 risk factors 4

Humbert et al. Am J Respir Crit Care Med 2006
72
Taken from the photo archive of Prof. Dr.
Müzeyyen Erk (2007)