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Pulmonary Sarcoidosis

Definition

  • Multisystem disease, usually involving the lung, characterized by noncaseating granulomas

Diagnostic Criteria

  • Lung is involved in >90% of cases
    • Other organs include heart, eye, kidney, liver, skin
  • Noncaseating granulomas
    • Tight, well-formed granulomas
      • Composed of epithelioid histiocytes and/or multinucleated giant cells
      • Sharply circumscribed from surrounding normal lung
    • Generally non-necrotizing granulomas
      • Small foci of necrosis can be seen, but no caseation (area of acellular necrosis surrounded by histiocytes and giant cells)
        • It is especially important to rule out infection in such cases
    • Mild to moderate surrounding inflammation at most
      • Usually "naked granulomas"
    • Inclusions frequently seen in giant cells (supportive but not diagnostic of sarcoid)
      • Asteroid bodies
        • Central core with radiating spikes
        • Not birefringent
        • 5-30 microns
      • Schaumann/conchoid bodies
        • Lamellar calcifications
        • Largely calcium carbonate, may polarize
        • 0.2-1.8 microns
      • Hamazaki-Wesenberg bodies
        • Oval, yellow-brown bodies
        • Frequently appear to bud, simulating yeast
        • Not birefringent
        • 1-15 microns
        • Positive on GMS and AFB stains
      • Calcium oxalate
        • Irregular birefringent crystals
        • 20-150 microns
  • Lymphatic pattern of involvement is usually seen
    • Follows bronchovascular bundles, interlobular septa and pleura (late in disease)
    • Frequently involves submucosa of bronchioles and bronchi
      • Results in high yield on bronchoscopic biopsy
      • May compress the airway lumen
  • With increasing age of lesions, fibrosis may become dominant
    • Concentric hyalinizing fibrosis around granulomas
    • May compress airways and vessels
    • Predominantly involves upper and mid lung fields
    • May rarely progress to honeycomb change or cavitation
  • Vasculitis seen in over half of cases
    • Infiltration of adventitia and media of arteries and veins by any or all of the following:
      • Granulomas, giant cells, lymphocytes and plasma cells
    • May lead to obstruction and pulmonary hypertension
  • Rare cases present as nodular sarcoidosis
    • Confluent nodule of granulomas
    • Usually 1-5 cm
    • Usually multiple
  • Very rare cases show necrotizing sarcoid granulomatosis
    • Variable amounts of necrosis in the presence of sarcoid-like granulomatous pneumonitis and granulomatous vasculitis
    • Usually multiple nodules 1-5 cm
    • It is especially important to rule out infection
  • Localized sarcoid-like reactions can be seen adjacent to neoplasms and other lesions and in draining nodes
    • True sarcoidosis is not focal
  • Sarcoidosis is a diagnosis of exclusion; the following must be ruled out in every case
    • Infection, especially fungi and mycobacteria
    • Hypersensitivity pneumonitis
    • Collagen vascular disease
    • Drug reaction
    • Metals/minerals reaction
      • Beryllium and rarely aluminum or talc

Gerald J Berry MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting/updates: 11/20/10

Differential Diagnosis

The following must be ruled out in every case

  • Infection
    • Fungi
      • Stain and culture
      • Usually more associated inflammation
    • Tuberculosis
      • Stain and culture
      • Usually caseous necrosis
      • Usually more associated inflammation
    • Mycobacteria avium intracellulare
      • Stain and culture
      • Usually non-lymphatic pattern, predominantly peribronchial
  • Reaction to metals/minerals
    • Berylliosis may appear identical to sarcoidosis
      • Must be excluded by history
    • Aluminum and talc may appear identical to sarcoidosis
      • Must be excluded by history
  • Hypersensitivity pneumonitis
    • Usually loose granulomas with more inflammation
    • Must be excluded by history
  • Local sarcoid-like reactions
    • Seen in tissue adjacent to neoplasms and in draining nodes
    • Sarcoidosis typically diffusely involves the lung
  • Wegener granulomatosis
    • Necrosis is suppurative with neutrophils
    • Necrosis surrounded by palisaded histiocytes

Clinical

  • Most patients 20-40 years old
  • Presentation ranges from asymptomatic to dyspnea and cough, with or without fever and weight loss
  • May spontaneously resolve
  • Usually steroid sensitive
  • End stage lung cases may be treated by transplantation
    • Disease may recurr in transplant but is rarely symptomatic

Report

  • An unequivocal diagnosis of sarcoidosis cannot be made histologically
  • Appropriate terminology for a report could be:
    • "Noncaseating granulomas, see comment"
    • The comment may state that the findings are consistent with sarcoidosis, but that infection, hypersensitivity pneumonitis, reaction to inhaled minerals and metals (beryllium, aluminum, talc) or drugs must be ruled out
    • Results of stains and cultures should be given, with the warning that they are not 100% sensitive

Classification / Lists

Idiopathic Interstitial Lung Diseases

Other Diffuse Parenchymal Lung Diseases

 

Bibliography

  • Travis WD, Colby TV, Koss MN, Rosado-de-Christenson ML, Müller NL, King TE Jr.  Non-neoplastic Disorders of the Lower Respiratory Tract, AFIP Atlas of Nontumor Pathology, First Series, Fascicle 2, 2002.
  • Ma Y, Gal A, Koss MN. The pathology of pulmonary sarcoidosis: update. Semin Diagn Pathol. 2007 Aug;24(3):150-61.
  • Thomas KW, Hunninghake GW. Sarcoidosis. JAMA. 2003 Jun 25;289(24):3300-3.
  • Shigemitsu H, Nagai S, Sharma OP. Pulmonary hypertension and granulomatous vasculitis in sarcoidosis. Curr Opin Pulm Med. 2007 Sep;13(5):434-8.
  • Ro JY, Luna MA, Mackay B, Ramos O. Yellow-brown (Hamazaki-Wesenberg) bodies mimicking fungal yeasts. Arch Pathol Lab Med. 1987 Jun;111(6):555-9.
  • Mochizuki I, Fukushima M, Hirose Y, Kono H, Gomi E, Suzuki K, Yokoyama N, Honda T, Hosokawa Y, Kuroiwa Y, et al. Ultrastructural studies of concentric lamellar bodies in the bronchus of patients with sarcoidosis. Sarcoidosis. 1987 Sep;4(2):111-5.
  • Pérez-Guillermo M, Sola Pérez J, Espinosa Parra FJ. Asteroid bodies and calcium oxalate crystals: two infrequent findings in fine-needle aspirates of parotid sarcoidosis. Diagn Cytopathol. 1992;8(3):248-52.
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