Sarcoidosis: An Encounter with a Granulomatous Disease

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Sarcoidosis An Encounter with a Granulomatous
Disease

• Barry Fanburg,MD
• Professor of Medicine
• Tufts University School of Medicine

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Disclosures

• No financial relationships to disclose.

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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