Title: CASE PRESENTATION
1CASE PRESENTATION
Prof. Dr. Birsen MUTLU I.U. Cerrahpasa Medical
Faculty Department of Pulmonology
2H. B.
- 56 years-old
- Female
- Born in Trabzon, lives in Istanbul
- Housewife
- Married
3COMPLAINTS
- Dyspnea
- Dry cough
- Weakness
- Swelling legs
- Dry mouth
- Dry eyes
4HISTORY
- 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.
5- HISTORY Hypertension (15 years)
- Diabetes mellitus Type 2 (5 yrs)
-
- FAMILIAL HISTORY Nothing special
- Addiction None
- Allergy Undefined
6- 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
7PHYSICAL EXAM - 1
- Conscious, cooperative
- Dyspnea, orthopnea ()
- Body temp37 ºC
- Obese
- Cyanosis (-)
- Palpable lymphadenomegaly (-)
- Clubbing (-)
- Raynauds phenomenon (-)
8PHYSICAL 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
9PHYSICAL 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
10PRE - DIAGNOSIS ???
- Pulmonary Hypertension
- Left Heart Failure
- Interstitial Lung Disease
- CTELD
- Cor Pulmonale
11CHEST X-RAY
H.B. 04. 06. 2007
12PRE - DIAGNOSIS ???
- Pulmonary Hypertension
- Left Heart Failure
- Interstitial Lung Disease
- CTELD
- Cor Pulmonale
13LABORATORY FINDINGS
14HAEMOGRAM
- WBC 9200/mm³
- Hb 12,2 gr/dL
- Hct 36,5
- PLT 259000/mm³
15- Sedimentation 38 mm/h
- CRP 21,5 mg/L
- BCG Scar 2 scars
- PPD 11 mm enduration
- Blood Biochemistry Normal
- Comp Urine Count Normal
16WHAT WOULD YOU FIRST PROPOSE FOR DIAGNOSIS ???
- Computerized Tomography
- Lung Function Tests
- TTD Ecocardiography
- ECG
- Serological Examination for Vasculitis
17COMPUTERIZED TOMOGRAPHY
k
l
18COMPUTERIZED TOMOGRAPHY
rv
ra
19COMPUTERIZED TOMOGRAPHY
rv
ra
20COMPUTERIZED TOMOGRAPHY
21HRCT
22HRCT
23ECG
24TT DOPPLER ECHOCARDIOGRAPHY
- Right cardiac spaces are extended
-
- Left ventricle EF 60
- Moderate tricucpid insufficiency
- Pulmonary arterial peak systolic pressure 85
mmHg
25RIGHT HEARTH CATHETERIZATION
- PULMONARY ARTERIAL PRESSURE
- Before adenosin 75/38 mmHg,
- mean 50 mmHg
- After adenosin 60/38 mmHg,
- mean 45
mmHg -
Vasoreactivity negative
26PRE - DIAGNOSIS ???
- Pulmonary Hypertension
- Left Heart Failure
- Interstitial Lung Disease
- CTELD
- Cor Pulmonale
27SPIROMETRE
28SPIROMETRE
29LUNG VOLUMES
30DIFFUSION CAPACITY
31ARTERIAL BLOOD GASES
- PO2 7O,8 mmHg
- PCO2 40,5 mmHg
- SaO2 94,2
- PH 7,46
- HCO3 28,9 mmol/L
- ? Room condution
32PULMONARY 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
33CLASSIFICATION OF PULMONARY HYPERTENSION(Simonnea
u et al. J Am Coll Cardiol 2004)
346DYT FUNCTIONAL CLASS
- 6DYT
- Distance 240 m
- SaO2 93 - 87
- Borg scala dyspnea 3-9
- NYHA IV
35ROMATOLOGICAL 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
36 SCHIRMERS TEST
- Right eye 20 mm
- Left eye 17 mm
- Normal
37WHAT WOULD YOU FIRST PROPOSE FOR DIAGNOSIS ???
- A. BAL
- B. Transbronchial Biopsy
- C. Salivary Gland Biopsy
- D. Open Lung Biopsy
- E. Transthoracic Biopsy
38LUNG 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
39WHAT 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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44DIAGNOSIS
SJÖGRENS SYNDROME
45SJÖGRENS 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
46SJÖGRENS 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
47CLINICAL 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
30
Harrisons principles of internal medicine. 16th
ed. 2005. 1990-1993
48LUNG 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
49CRITERIA 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.
50CLINICAL 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
51CLINICAL 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
52CHEST X-RAY
H.B. 11.01.2008
53PA GRAFILER
11.01.2008
04.06.2007
54SPIROMETRY
04.06.2007
11.01.2008
55DIFFUSION CAPACITY
04.06.2007
11.01.2008
- DLCO 9,04 (48) mL/mmHg/min
- DLCO/VA 3,35 (67) mL/mmHg/min/L
56ARTERIAL 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
57WHAT WOULD YOU THINK AT THIS POINT ???
- The patient should be followed up
- Immunsuppresive dose should be increased
- Treatment for PH should be started
- Another factor should be looked for
- The disease is progressing
582. COMPUTERIZED TOMOGRAPHY
592. COMPUTERIZED TOMOGRAPHY
602. HRCT
61WHAT WOULD YOU THINK AT THIS POINT ???
- The patient should be continued to be followed
- Immunsuppresive dose should be increased
- Treatment for PH should be started
- Another factor should be looked for
- The disease is in progress
622. 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)
632. RIGHT HEART CATHETERISATION
- Pulmonary arterial pressure 77/35 mmHg
(average 50) -
- Atrial septal defect with shift from left to
right!!! - Transesophageal Echocardiogram proposed
64TRANSESOPHAGEAL 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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66ATRIAL 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
67ATRIAL 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
68ATRIAL 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
69ATRIAL 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
70ATRIAL 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
71PH TYPES WHEN DIAGNOSED
Portal hypertension
Humbert et al. Am J Respir Crit Care Med 2006
72Taken from the photo archive of Prof. Dr.
Müzeyyen Erk (2007)