clinical pathology conference

7/31/2019 Clinical Pathology Conference

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Clinical Pathology Conference

42 yo man with recurrentpneumonia

J. R. Hartig, MD

November 28, 2006


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CPC: Goals

Its all about the discussion(If Im wrong!)

Its all about being correct(If the discussion is bad)

Its all about having the diagnosis in thedifferential

(My DDx will include all of Harrisons) Its all about getting even with the CMRs

(If this is something totally ridiculous)


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CPC: Goals

Discuss the approach that I used in thiscase

Discuss some uncommon pulmonaryconditions

Make an educated guess after narrowing

the differential


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Start from the start

42 yo white male with blood-tingedproductive cough


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Differential Diagnosis

Common things are common

Bronchitis

Viral URI

Rhinosinusitis (Allergies)

Pneumonia

GERD


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Start from the start

42 yo white male with blood-tingedproductive cough

Recurrent pneumonias

Several CAP during the past year


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Missouri State Motto


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Pulmonary

Local

Diffuse

Immune

Gastrointestinal Problems

Neurological Conditions


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Pulmonary

Local

Anatomic abnormality Bronchial compression

LAD

Neoplasm

Vascular anomaly

Foreign body Bronchiectasis

Bronchomalacia / stenosis

Tracheo-esopahgeal or bronchial fistulas


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Pulmonary

Diffuse recurrence

Underlying pulmonary process Cystic fibrosis

Immotile cilia syndromes

Neurological dysfunctions

Dysphagia Seizures

Etoh/drugs


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Cystic Fibrosis

Autosomal recessive disease

1:2000 to 3000

CFTR protein abnormality

Multi-organ disease

mild mutations

Productive cough

No hint of disease earlier in life


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Pulmonary

Diffuse recurrence

Underlying pulmonary process Cystic fibrosis

Immotile cilia syndromes

Neurological dysfunctions

Dysphagia Seizures

Etoh/drugs


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Immune

Quantitative or Qualitative granulocytic

Chronic Granulomatous Disease

Multiple Myeloma

Common Variable Immune Deficiency

Chronic lymphocytic leukemia HIV


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Gastrointestinal Problems

Aspiration (primary or secondary)

GERD

Zenkers diverticula

Achalasia

Neurologic Conditions


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More History

Chronic non-productive cough

Progressive dyspnea over the coarse of a

year

No fevers, chills, night sweats

Weight loss >20lbs

Antibiotics give short term relief

Steroids do not seem to help


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Some of the teasers

Works as a hairstylist

+MSM

27 pk/yr tobacco use

No drugs, No Etoh, No TB risks (?homeless)

ROS: no other signs of bleeding problems


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Exam

Marked hypoxia on room air (80%)

Chronically hypoxic (clubbing)

Pulmonary exam

Mild tachypnea

Bibasilar rales


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Clubbing


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Labs

Marked A-a gradient

Elevated bicarb

Hct 49

LDH 603

WBC 19K But not really lymphopenic

Numerous other negative labs


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Additional Testing

Normal Transthoracic echocardiogram

CXR and CT

Patchy ground glass opacities diffusely with ahilar predominance. No lymphadenopathy.


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Lucy!!! You got some splaining to

doYoung W chronic non-productive cough

Recurrent Pneumonias

Severe Dyspnea

Weight Loss

Antibiotics some help. Prednisone none. Chronic hypoxia

Hilar predominant radiographic changes


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Where to now?

Infections

Vascular

Malignancy

Pulmonary

Interstitial Lung Diseases (DiffuseParenchymal Lung Diseases)

Diffuse Alveolar Hemorrhage Syndromes


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Infections (Pneumonia)

Previously healthy host

Diagnosed with pneumonia

Elevated WBC (left shift)

Gets better with antibiotics


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Infections (Pneumonia)

Viral

Bacterial Nocardia

Mycobacterial M. tuberculosis

Non-tuberculous Mycobacterial infections

Fungal

Histoplasmosis Coccidioidomycosis

Other/Parasitic Pneumocystis jiroveci


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Nocardia

Aerobic gram + bacteria

16 species causing human disease

Immunocompromised hosts Pulmonary manifestations

Non-improving pneumonia

TB mimic on x-ray

Diagnosis Treatment

Sulfonamides


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Tuberculosis

Common in Alabama

Always in the differential on a CPC

No risk factors

? Homeless

PPD (-)

No hilar adenopathy

Too many other things are possible


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Nontuberculous Mycobacteria

M. avium and M. intracellulare (MAC)

Older individuals with COPD

Bronchiectasis (cystic fibrosis)

Clinically similar to TB

M. kansasii

Similar to TB; cavitation common

Diagnosis and Treatment


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Histoplasmosis

First described in 1906 Common among Ohio and Mississippi River

valleys Most individuals have few symptoms Pulmonary disease

Bronchopneumonia Hilar LAD

Diagnosis Urine 75% pulmonary disease Serology 90% acute pulmonary disease


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Histoplasmosis


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Pneumocystis carinii (P. jiroveci)

pneumonia HIV (-) individuals

Malignancy

Hematologic > solid

Steroids

Mean dose 30mg/d and 12 weeks therapy

Often seen after therapy is stopped

Diagnosis and Therapy


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Congenital

Cystic fibrosis (better not be)

Infections PCP


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Where to now?

Infections

Vascular

Malignancy

Pulmonary

Interstitial Lung Diseases (Diffuse

Parenchymal Lung Diseases)

Diffuse Alveolar Hemorrhage Syndromes


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Vascular/Vasculitis

No obvious bleeding abnormality

No specific evidence to indicate PTE or

pulmonary veno-occlusive disease

Wegeners granulomatosis

Churg-Strauss syndrome

Microscopic polyangiitis


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Lungs and kidneys

25% of cases have only respiratory

involvement Primarily have sinus involvement

90% antineutrophil cytoplasmic antibodies

X-ray usually demonstrate nodules or LAD


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Vasculitis

Churg-Strauss syndrome

Usually starts with asthma (8-10 years)

Skin and nasal disease is common No specific labs: eosinophilia, anca, others

Microscopic polyangiitis

Also has predominance of anca (+)


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Congenital


Infections PCP

Vasculitis

?


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Malignancy

Lymphoma

Bronchogenic carcinoma


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Congenital


Infections PCP

Vasculitis/Malignancy

?


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Where to now?

Infections

Vascular

Malignancy Pulmonary

Interstitial Lung Diseases (Diffuse

Parenchymal Lung Diseases) Diffuse Alveolar Hemorrhage Syndromes

Other


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Interstitial Lung DiseaseDiffuse Parenchymal Lung Disease

Idiopathic Pulmonary Fibrosis (UIP)

Other Idiopathic Interstitial Pneumonia

Desquamative IPAcute IP

Nonspecific IP

Respiratory bronchiolitis assoc -ILD

Cryptogenic Organizing Pneumonia

Known causes (Hypersensitivity, CTD)


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Idiopathic Pulmonary Fibrosis

Present in 5th or 6th decade of life

Progressive dyspnea and non-prod cough

: of 2:1 Elevated LDH

Follows relentless coarse difficult to treat

Radiographically Peripheral and bibasilar reticulonodular

opacities with traction bronchiectasis


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Desquamative IP


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Desquamative IP


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Respiratory bronchiolitis assoc -ILD


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Clinically presents in the 5th to 6th decade

Idiopathic form of BOOP

Persistent non-productive cough

Dyspnea

Weight loss (10lbs average)

Steroids help; antibiotics generally do not


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Hypersensitivity Pneumonitis

Certainly could explain the radiographicfindings

Lack of specific agent (work exposure?) Chronic recurrent exposure

Generally improves with corticosteroids


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Congenital


Infections

PCP

Vasculitis/Malignancy ?

DPLDs


Desquamative Interstitial Pneumonia


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Diffuse Alveolar HemorrhageSyndromes

Anti-GBM disease (Goodpastures)

Idiopathic Pulmonary Hemosiderosis

Hct is normal

UA is normal


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Other

Sarcoidosis

Pulmonary Langerhans cell histiocytosis

Pulmonary Alveolar Proteinosis


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Sarcoidosis

Worldwide disease young adults

Multisystem granulomatous disorder

Characteristics: Bilateral hilar adenopathy

Pulmonary infiltrates

Skin/eye abnormalities Treatment

Generally involves steroids


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Pulmonary Langerhans cellHistiocytosis

Younger smoking patients (20-30s)

Caucasian

Fever, weight loss, other symptoms Spontaneous pneumothorax

Variable clinical coarse

PFTs Reticular nodular infiltrates and honeycombing

in upper lung zones


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Accumulation of lipoproteinaceous in thedistal airways

No inflammation or architectural changesAlveolar macrophage dysfunction

Impaired response to GM-CSF

Increased risk of superinfections


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Clinical Presentation

Typically age 30-50

Male to female 2:1 Insidious onset

Symptoms

Progressive dyspnea on exertion

Fatigue

Weight loss

Non-productive cough is common


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Radiographic findings

Bat-wing distribution

Air bronchograms are rareCrazy paving


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Laboratory findings:

Polycythemia

Increased LDH Hypergammaglobulinemia

Marked hypoxia

serum anti-GM-CSF titer elevation


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Diagnosis

Bronchoalveolar lavage or biopsy

Treatment Whole lung lavage

Prognosis

??


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Congenital


Infections

PCP

Vasculitis/Malignancy ?

DPLDs Idiopathic Pulmonary Fibrosis

Desquamative Interstitial Pneumonia

DAH Sarcoid

PAP


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Final Answer

Procedure

Bronchoscopy with BAL and TBBx

Diagnosis Pulmonary Alveolar Proteinosis

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