Clinical Pathology Correlate
Conference
Katrina Cannon, MD
Internal Medicine
Case Presentation
50 yo African American female with 2 year hx
of intermittent dyspnea presented to ER with
marked SOB and respiratory distress
Pulmonary clinic 6 months prior for dyspnea
and abnormal CXR; recommended further
evaluation including CT; not completed
Significant dyspnea ~ 2 weeks new heart
palpitations
ER course
Physical Exam
Gen: significant respiratory distress, cool
but no diaphoresis, no pain
VS: HR 120-130 and BP 160/100
CV: distant heart sounds, no m/r
Lungs: poor breath sounds, expiratory
wheezes and “tightness”
Treatment: oxygen by facemask
Acute Respiratory Failure
ContinuedSOB, appeared hypoxic
ABG: pH 7.15, PaCO2 51, PaO2 200
Decreasingly responsive
Rapid Sequence Intubation
Sedation: versed, fentanyl
Paralyzed: succinylcholine,vecuronium
Intubation: ETT placed
ER course
Physical Exam
VS: BP 80/60
Extremities: mottled, cool, hypoperfused
EKG/CXR/labs obtained
EKG
CXR
Labs
Glucose 230
Na 130, K 3.6, Cl 106, HCO3 22
BUN 10, Cr 0.8
WBC 8.7 (54% bands, 10% lymphs),
Hgb 9.5, Platelets 347,000
CK 53, LDH 218, Trop T WNL
ER Course continues…
Hypotension tx: IVF and Dopamine
Cardiology Consult: abnormal EKG,
persistent hypotension and tachycardia
? possible AMI with cardiogenic shock
Cardioversion attempted-unsuccessful
Echocardiogram
Echocardiogram
Mild generalized hypokinesis
More focal wall motion in anterior apical
wall
EF mildly reduced- difficult to assess
due to tachycardia
LVH
No acute valvular disorders
Small pericardial effusion
ER Course continues…
Adenosine (transient AV block) revealed
underlying atrial rate of ~240
Digoxin initiated
Dopamine Neo-Synephrine started
BP responded, blood gas improved:
ABG pH 7.3 35/135 HCO3 17
Transferred to ICU
ICU Course: Day 1
ventilator
Respiratory:
Cardiovascular:
digoxin + procainamide
HR 100-110
atrial tachycardia persisted
Diuresedwith brisk response
Neo-Synephrine tapered off
PA Catheter
PCWP = 13 (12-18)
RA = 9 (2-6)
PAP = 46/32 (25/10)
CO = 5.4 (5.4 - 7.2)
CI = 2.9 (2.9 - 3.9)
PVR = 829 (200
known diseases
Non-malignant and non-
infectious
Diffuse Parenchymal Lung Disease
Known Cause: Idiopathic Granulomatous: Pulmonary Hemorrhage
Drugs/Treatment Interstitial Sarcoidosis Syndromes
Environmental Exposure Pneumonia PLCHistiocytosis LAM
Collagen Vasc Disease Amyloidosis
Idiopathic IIP
Pulmonary other than
Fibrosis IPF
Desquamative Respiratory Acute Lymphocytic Non-specific Cryptogenic
Interstitial Bronchiolitis Interstitial Interstitial Interstitial Organizing
Pneumonia ILD Pneumonia Pneumonia Pneumonia Pneumonia
(provisional) (BOOP)
Primary Disease associated
with Interstitial Lung Disease
Sarcoidosis
PLCH
Vasculitides
Hemorrhagic syndromes
Amyloidosis
Lymphangiolieomyomatosis (LAM)
Lymphangitic Carcinomatosis
Congenital/Hereditary
Chronic Pulmonary Edema
Alveolar Proteinosis
Pulmonary Veno-occlusive Disease
ILD: History
Timing: Chronic (months to years)
Idiopathic Pulmonary Fibrosis
Sarcoidosis
Pulmonary Langerhans Cell Histiocytosis (PLCH,
Histiocytosis X, Eosinophilic Granuloma)
Age (50)
Sarcoid, PLCH, CVD, LAM, inherited ~20-40
IPF >50
History continued…
Family History: negative
Neurofibromatosis
TuberousSclerosis
Hermansky-Pudlak syndrome
Occupational/Drug Exposure: none
no amiodarone
ACE induced cough
Procainamide ~drug-induced SLE
History continued…
Gender: LAM, tuberous sclerosis only in
premenopausal women
Race: African American
Sarcoidosis 3-4 times more common in blacks,
lifetime risk 2.4% v. whites 0.85%
Smoking History: non-smoker
+tob: PLCH, DIP, IPF, respiratory bronchiolitis
-tob: sarcoidosis, hypersensitivity pneumonitis
Physical Exam
Dyspnea, wheezing
No cough, no hemoptysis, no chest pain
BOOP/COP
Respiratory bronchiolitis
Hypersensitivity pneumonitis
Diffuse alveolar hemorrhage syndromes
Granulomatous vasculitides
Maligancy
No extrapulmonary findings
Labs
Normal electrolytes
Normal renal function
Mild Anemia, stable
ABG: respiratory acidosis-resolved
Labs continued…
ACE level 40 (7-46) upper limits of normal:
Increased 35-80% sarcoidosis
But can be normal (sensitivity 57%)
Less likely increased in chronic sarcoidosis
Also increased Silicosis, TB, LIP
No ANCA: Wegner’s granulomatosis
No Anti-BM ab: Goodpasture’s
syndrome
CXR/CT
6 months prior Current
ILD plus Perihilar Adenopathy
Sarcoidosis
Lymphoma
Lymphangitic carcinomatosis
Infectious: TB or Fungal Disease
(histoplasmosis)
Lymphocytic interstitial pneumonia
Amyloidosis
Pneumoconioses (Beryllium, Silicosis)
Bronchiogenic Carcinoma
Sarcoidosis
Evidence For Evidence Against
Race: African American CXR/CT: Lower lung
Age:
lower)
Stage III: interstitial
disease with shrinking
hilar nodes
Stage IV: advanced
fibrosis
Sarcoidosis
Evidence For Evidence Against
Race: African American CXR/CT: Lower lung
Age: <50 zone
CXR/CT:Bilateral Bronch: stenosis
Pulmonary infiltrates and
perihilar adenopathy Lab: ACE ?
PFTs: restrictive, DLCO No extrapulmonary
Bronch: endobronchial manifestations…
lesions
CV: tachyarrhythmia
Sarcoid and Cardiovascular Disease
Not uncommon:
5% clinically significant
25-30% postmortem
Granulomatous
infiltration
Conduction
disturbances
Atrial Tachycardia*
Conclusion
50 yo AAF with Chronic Dyspnea
Pulmonary Sarcoidosis
Focal Atrial Tachycardia ~ CV sarcoid
Acute Respiratory Failure:
CHF/Pulmonary Edema
Cardiac: diuresis and antiarrhythmics
Tx: Steroids
Dx: Sarcoidosis
Clinicaland Radiographic findings
supportive of Sarcoidosis
R/O infection:TB and fungal disease
Histologic evidence of noncaseating
granulomas
Diagnostic Result
Noncaseating Granulomas
SARCOIDOSIS
The End
1.Proceed to the post test
2. Download the post test
3. Complete the post test
4. Return the post test to Dr. S. Oliver
407i TAMUII
Post test question 1
Sarcoidosisis most often diagnosed in
which of the following EXCEPT:
Young adults (20-40 years old),
Nonsmokers
Hispanic-Americans
Roughly equal in Caucasian men and
women
Post test question 2
Sarcoidosis is usually treated initially
with which of the following:
1. Steroids
2. Methotrexate
3. Thalidomide
4. Cytoxin
References
ACC/AHA/ESC Guidelines for the Management of Patients with
Supraventricular Arrhythmias; JACC Vol. 42. No. 8, 2003, Oct
15 2003: 1513-1516.
Braunwald, E; Essential Atlas of Heart Disease; Current
Medicine 1997; Philadelphia, PA; pg 5.9.
Colucci, W; Braunwald, E; Atlas of Heart Failure: Cardiac
Function and Dysfunction; 2nd Ed; Current Medicine 1999;
Philadelphia, PA; pg 3.11.
Schwarz, M; King, T; Interstitial Lung Disease 4th Ed.; BC
Decker Inc.; Hamilton London; 2003.
Uptodate: Approach to the Adult with Interstial Lung Disease,
Evaluation of Diffuse Lung Disease by Plain Chest Radiography,
Overview of Sarcoidosis, Atrial Tachycardias