Johns Hopkins CPC

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Johns Hopkins CPC4

• David R. Moller, M.D.
• Johns Hopkins University
• Baltimore, USA

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General Approach to Difficult to Manage
Sarcoidosis

• Biopsy never diagnostic.
• Always revisit diagnosis or consider additional
  pathologic processes when either clinical course
  or clinical manifestations deviate from the
  expected or the typical.
• Consider
• Not sarcoidosis
• Sarcoidosis plus independent process-e.g.
  infection, PE, CHF
• Sarcoidosis plus associated process- e.g. CVID,
  CTD, others
• Treatment effects
• Rare manifestations of sarcoidosis do occur

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Asymmetric infiltrates not pulm sarc
Bilateral gg-not pulm sarc with tx.
Denser peripheral infiltrates, air bronchograms
prob pneumonia
? More bronchiectasis, cystic lesions on
L--?structural abn
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Matted extensive anterior/middle mediastinal LA
very unusual distribution, asym for sarc-- !
Assume not sarcoidosis
Big SVC, Big PAs, c/w pulm htn
Hilar LA and central infiltrates Narrowed,
thickened bronchi
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Air bronchograms
No med shift,atelectasis
Med-widened ?LNs only
Prob RV enlargement-?cardiac sarc
Cavity?
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Clues from the chest CT and radiograph

• Possibly explained by multisystem sarcoidosis
• RV enlargement from pulmonary htn (?cardiac sarc)
  
• Bilateral hilar lymphadenopathy mild ILD
• Bronchiectasis
• Not explained by pulmonary sarcoidosis
• Bilat ground glassnot pulm sarcoidosis on Pred
  20mg/d, ?infection ?CHF
• Denser L pulm infiltrate acute/subacute
  pneumonia
• More extensive L bronciectasis/cystic changes
  suggest possible chronic process ?bronchial
  stenosisbut no atelectasis, no obstructive
  impairment
• Left sided paraaortic upper mediastinal
  lymphadenopathy-generalized LN Not consistent
  with sarcoidosis esp with tx
• matted LN mass histo,TB, other fungal gtlymphoma
  

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Differential Diagnosis of Granulomatous Lung
Disease
Non-infectious
Infectious

• Sarcoidosis
• Chronic beryllium disease
• Hypersensitivity pneumonitis
• Wegeners granulomatosis
• Churg-Strauss Syndrome
• Necrotizing sarcoid granulomatosis
• Lymphoma
• Lung cancer, other metastatic cancer
• Lymphomatoid granulomatosis
• Crohn disease
• Pneumoconiosis (silicosis)
• Common variable immunodeficiency
• Blau syndrome

• Mycobacterial
• Fungalhisto, blasto, cocci, etc
• Protozoaltoxoplasmosis
• Spirochetal- T. pallidum
• Bacterial-brucella, yersinia

Misc.

• Bronchiolitis obliterans organizing pneumonia
  (BOOP)
• Lymphocytic interstitial pneumonitis
• Sjogrens syndrome

show typical compact epithelioid granulomas
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What is the differential diagnosis of
granulomatous inflammation in the lung in this
patient?

• Differential diagnosis of bilateral hilar and
  mediastinal lymphadenopathy plus interstitial
  lung disease
• Surgical lung bx should reasonably exclude
  noninfectious granulomatous lung disease e.g.
  Wegener granulomatosis, hypersensitivity
  pneumonitis, cryptogenic organizing pneumonia
• Never can completely exclude infection e.g.,
  mycobacterial, fungal disease or (rarely)
  malignancy e.g., lymphoma
• Look for extrapulm manifestations of sarcoidosis
  to confirm diagnosis

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Sarcoidosis Associated Pulmonary Hypertension

• Pulmonary hypertension prevalence
• 6 unselected pulm sarcoidosis patients
• 50 patients with dyspnea disproportionate to
  PFTs
• 70-80 in advanced lung disease
• Higher when measuring exercise induced pulm htn
• Multiple potential mechanisms
• Bronchovascular distribution of inflammation
• Advanced fibrocystic lung disease (loss of
  pulmonary capillary bed)
• Extrinsic compression of pulmonary arteries by
  LN, mediastinal fibrosis
• Cardiac sarcoidosis with systolic, diastolic
  dysfunction
• Hypoxic vasoconstriction
• Primary pulmonary vascular involvement
  (granulomatous arteritis)
• Veno-occlusive disease (rare)

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What is the most likely cause of her pulmonary
hypertension?

• Common causes of pulmonary hypertension in
  sarcoidosis
• Sleep apnea (mild pulm htn)
• Advanced interstitial lung disease (stage 3, 4
  fibrocystic sarc)
• Chronic pulmonary embolism (must rule out)
• L heart failure ( diastolic dysfunction, cardiac
  sarcoidosis)
• Pulmonary vascular sarcoidosis
• Interstitial lung disease does not explain pulm
  htn
• Diffusing capacity does not correlate with pulm
  htn
• DLCO decreased in pulm htn secondary to
  fibrocystic pulm sarcoidosis

Note these same causes may be present in
infectious granulomatous diseases
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Cardiac Sarcoidosis Clinical Manifestations

• Common
• Arrhythmias
• Heart block/conduction defects
• Congestive heart failure
• Sudden death

• Rare (lt10 all cardiac sarcoidosis)
• R ventricular involvement
• Valvular dysfunction
• Pericarditis
• Myocardial mass
• Coronary vessel involvement

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What are the possible causes of the patients
worsening shortness of breath over months and
eventual demise??

• Chronic slowly progressive dyspnea possibly
  explained by multisystem sarcoidosis with
• Interstitial lung disease (in part)
• Pulmonary hypertension- secondary pulm
  arterial/arteriolar involvement
• ? cardiac sarcoidosis
• Treatment unresponsiveness ? Suspect pulm htn and
  fibrosis
• Worsening dyspnea over months not explained by
  sarcoidosis
• Left sided infiltrates/pneumonia
• L sided mediastinal lymphadenopathy ?new

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What are the possible causes of the patients
worsening shortness of breath over months and
eventual demise?

• Multisystem sarcoidosis infection
• pulmonary (ILD)BHA plus LgtR bronchiectasis
• pulmonary hypertension from pulm vascular
  involvement-?prox or distal arterial possible
  cardiac involvement
• L sided infiltrates Secondary CAP or HAP
  (?bronchial stenosis, rule out CVID)
• L mediastinal LA ? secondary to histoplasmosis,
  mycobacterial, other fungal infection or nocardia
  which could also explain L pneumonia
• Infectionchronic histoplasmosis vs tuberculosis
• Infiltrates, mediastinal lymphadenopathy, tongue
  papule?, pulm htn from pulm artery involvement
  from med/hilar LN, fibrosis
• Lymphoma sarcoidosis plus infection

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What are the possible causes of the patients
worsening shortness of breath over months and
eventual demise?

• PEA arrest
• Pulmonary - Respiratory arrest with hypoxia
• Mechanical
• tension pneumothorax fibrocystic lesions
• cardiac rupture with severe CHF (papillary
  muscles, aneursym)
• cardiac tamponade pericarditis
• Preload and afterload changes (severe pulm htn)
• pulmonary embolus
• sepsis (pneumonia, ?mediastinitis)
• Metabolic changes no evidence
• Note if arrhythmia, heart block at CP arrest,
  suspect cardiac sarc