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Sarcoidosis: An Encounter with a Granulomatous Disease

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Title: Sarcoidosis: An Encounter with a Granulomatous Disease


1
Sarcoidosis An Encounter with a Granulomatous
Disease
  • Barry Fanburg,MD
  • Professor of Medicine
  • Tufts University School of Medicine

2
Disclosures
  • No financial relationships to disclose.

3
Objectives
  • Explain the pathogenesis.
  • Evaluate features of and diagnostic approaches to
    sarcoidosis.
  • Summarize options for therapy.

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New Articles
  • Iannuzzi MC et al, Sarcoidosis (Review) NEJM 357
    2153-2165,2007.
  • Lynch JP et al, Pulmonary Sarcoidosis, Semin
    Respir Crit Care Med 28 053-074,2007.
  • Baughman RP, Lower EE, Evidence-based Therapy for
    Cutaneous Sarcoidosis (Review). Clinical Derm
    25(3) 334-340,2007.

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Dates of Importance for Sarcoidosis
  • 1951- First use of corticosteroid in treatment
  • 1975- Fiberoptic bronchoscopy first used led to
    biopsy and cytolology (T lymphocytes in
    pathogenesis)
  • 1975- Recognition of ACE elevation

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Cause ?????
  • Environmental
  • Genetic

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Symptoms and Signs in Presentation of Sarcoidosis
  • Asymptomatic
  • Cough,dyspnea,wheezing,chest pain
  • Clubbing and hemoptysis rare
  • Remarkably normal auscultation of chest

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Non-Specific Manifestations of Sarcoidosis
  • Fatigue
  • Fever
  • Chest pain

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Stages of Pulmonary Sarcoidosis
  • 0- No intrathoracic finding
  • 1- Bilateral hilar adenopathy
  • 2- Bilateral hilar adenopathy plus parenchymal
    infiltrates
  • 3- Parenchymal infiltrate only
  • 4- Honeycombing,hilar retraction, bullae,
    cysts,emphysema (pulmonary fibrosis

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Chest CT Findings in Sarcoidosis
  • Widespread small nodules with bronchovascular and
    subpleural distribution
  • Thickened interlobar septae
  • Architectural distortion
  • Conglomerate masses
  • Honeycombing
  • Cyst formation and bronchiectasis
  • Alveolar consolidation

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PET Scanning in Sarcoidosis
  • Tierstein A et al, Whole body flurodeoxyglucose
    positive emission tomography in sarcoidosis,
    Chest 2007 (E pub ahead of print).
  • 188 PET scans in 137 patients.
  • Most common in lymph nodes, some occult sites
    stage II and III mostly.

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Ancillary Laboratory Studies to be Considered in
Sarcoidosis
  • CBC
  • Serum calcium
  • Urinary calcium
  • LFTs
  • Cr/BUN
  • Serum ACE
  • EKG

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Involvement of Nasopharynx and Upper Airways in
Sarcoidosis
  • Nose
  • Sinuses
  • Larynx
  • Trachea

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Specific Cutaneous Lesions of Sarcoidosis
  • Plaques/nodules
  • Lupus pernio
  • Infiltrated scars
  • Maculopapular
  • Ichthyosiform
  • Hypo- or hyperpigmented
  • Erythrodermic
  • Ulcerative
  • Multilatory
  • Psoriasiform
  • Lupus erythematosus-like
  • Scarring alopecia
  • Nail changes
  • Subcutaneous
  • Mucosal
  • Atrophic

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Presentation of Neurosarcoidosis
  • Perhaps 5-15 of all sarcoidosis
  • Neurologic presentation may come first
  • Neurologic may be only feature
  • More often other sarcoidosis findings are
    associated

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Presentation of Neurosarcoidosis (contd)
  • Cranial nerve-facial, otic,opthalmic
  • Meningoencephalitis
  • Cranial space occupying lesion-seizures,headaches,
    focal symptoms,alteration in cognition
  • Hydrocephalus

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Presentation of Neurosarcoidosis (contd)
  • Spinal cord lesion,cauda equina presentation
  • Pituitary-panhypopituitarism,diabetes insipidus
  • Mono or poly-neuropathy
  • Phrenic nerve paralysis,respiratory failure
    (rare)
  • Cerebellar malfunction

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Calcium Elevation in Sarcoidosis
  • Hypercalcemia,hypercalcuria,nephrocalcinosis
  • May be associated with renal failure
  • 1,25-dihydroxyvitamin D increased (from
    granulomas)
  • Increased intestinal absorption of calcium and
    bone resorption
  • Exclude hyperparathyroidism

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Cardiac Findings in Sarcoidosis
  • Arrythmia,PVCs,V-tach
  • Conduction disturbance
  • Cardiomyopathy,CHF
  • Pericardial disease
  • Sudden death

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Sarcoidosis Can Mimic Cancer
  • Nodular lesions in lungs, brain and breast
  • Cystic bone disease
  • Lymph node enlargement

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Diagnosis of Sarcoidosis
  • What is needed ?
  • Typical clinical presentation
  • Tissue biopsy
  • Serum ACE
  • Kveim test, gallium scan, BAL ?

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Differential Diagnosis
  • Tuberculosis
  • Atypical mycobacterium
  • Fungal infection
  • Hypersensitivity pneumonia
  • Crack cocaine inhalation

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Differential Diagnosis (contd)
  • Drug reactions
  • Aspiration of foreign materials
  • Necrotizing sarcoid granulomatosis
  • Berylliosis
  • Alpha-interferon administration

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Course of Sarcoidosis
  • Spontaneous remission
  • Burn-out and scarring
  • Progression

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Decisions Regarding Therapy
  • Significant symptomatology-judgemental
  • Significant involvement of vital organs
  • May not treat a) Diffuse chest x-ray
    abnormality, b) Pulmonary function impairment

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Review of Therapy
  • Baughman,RP, Lower EE, Novel Therapies for
    Sarcoidosis, Semin Respir Crit Care Med 2007
    28128-133.

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Treatment of Sarcoidosis
  • None
  • Prednisone concepts Small dose, every other
    day, taper, possibly stop intermittently
  • Other therapies anti-inflammatory, eg
    chloroquine immunosuppressive, eg methotrexate,
    cytoxan, infleximab

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Use of TNFalpha inhibition in sarcoidosis tharapy
  • Sweiss NJ, Curran J, Baughman RP, Role of tumor
    necrosis factor inhibitors and other biologic
    agents, past, present and future concepts
    (Review), Clin Dermatol 25(3) 341-6,2007.
  • Kahler, CM et al, Infleximab therapy, Am J Respir
    Crit Care Med 176 (4) 417, 2007.

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Potential Side Effects of Prednisone Therapy
  • Weight gain and fluid accumulation
  • Bone demineralization and fractures
  • Increased succeptability to infection
  • Cataracts
  • Mood alterations
  • Diabetes
  • Hypertension
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