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Tuberculosis, often called TB, is a contagious illness primarily caused by Mycobacterium tuberculosis. It mainly affects the lung parenchyma but can also impact other body parts.
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Advanced Pulmonary Sarcoidosis.

Paolo Spagnolo1, Zehra Dhanani2, Paolo Cameli3

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Summary
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Advanced pulmonary sarcoidosis (APS) affects 20% of patients, causing fibrosis and complications. Management strategies are discussed due to the lack of natural history data and treatment guidelines for this severe lung condition.

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Area of Science:

  • Pulmonary Medicine
  • Immunology
  • Radiology

Background:

  • Pulmonary manifestations are common in sarcoidosis, with 20% progressing to advanced pulmonary sarcoidosis (APS).
  • APS is characterized by pulmonary fibrosis and complications like bronchiectasis, chronic pulmonary aspergillosis, and pulmonary hypertension.
  • The natural history and evidence-based treatment guidelines for APS are currently lacking.

Purpose of the Study:

  • To review the major patterns of advanced pulmonary sarcoidosis (APS).
  • To emphasize clinical manifestations, pathophysiology, and management strategies for APS.
  • To provide expert opinion on managing this severe lung disease phenotype.

Main Methods:

  • Review of existing literature and expert opinion.
  • Emphasis on clinical presentation and pathological mechanisms.
  • Discussion of current and potential therapeutic interventions.

Main Results:

  • Advanced pulmonary sarcoidosis (APS) presents with significant morbidity and mortality.
  • APS is often active, as indicated by 18F-fluorodeoxyglucose-PET scans.
  • Management may involve anti-inflammatory treatments, antifibrotics, pulmonary rehabilitation, antibiotics, vasodilators, and lung transplantation.

Conclusions:

  • Advanced pulmonary sarcoidosis (APS) is a severe phenotype requiring comprehensive management.
  • Treatment approaches are tailored to the predominant disease phenotype.
  • Further research into the natural history and evidence-based guidelines for APS is crucial.