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Sarcoidosis

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Updated 2024 AAFP guidelines for the diagnosis and management of sarcoidosis.

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of sarcoidosis are prepared by our editorial team based on guidelines from the American Academy of Family Physicians (AAFP 2024), the American College of Rheumatology (ACR 2023), the European League Against Rheumatism (EULAR 2023), the European Society of Cardiology (ESC 2023,2021), the European Respiratory Society (ERS 2022,2021), the American Thoracic Society ...
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Screening and diagnosis

Diagnosis: as per AAFP 2024 guidelines, implement a holistic approach for the diagnosis of sarcoidosis, focusing on clinical presentation and imaging findings, biopsy with evidence of noncaseating granulomas, involvement of more than one organ system, and excluding other etiologies of granulomatous disease.
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Classification and risk stratification

Staging: as per ATS/ERS/WASOG 1999 guidelines, use the following radiographic staging for sarcoidosis:
Situation
Guidance
0
Normal CXR
I
Bilateral hilar lymphadenopathy
II
Bilateral hilar lymphadenopathy plus pulmonary infiltrations
III
Pulmonary infiltrations without bilateral hilar lymphadenopathy
IV
Pulmonary fibrosis
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Diagnostic investigations

Initial laboratory tests: as per ATS 2020 guidelines, consider obtaining a complete blood cell count in patients with sarcoidosis, in order to screen for hematological abnormalities.
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  • Evaluation for pulmonary hypertension

  • Evaluation for cardiac involvement (ECG and TTE)

  • Evaluation for cardiac involvement (MRI and PET)

  • Evaluation for renal sarcoidosis

  • Evaluation for hepatic sarcoidosis

  • Evaluation for ocular involvement

  • Screening for chronic infections

Diagnostic procedures

Bronchoalveolar lavage: as per BTS/ITS/TSANZ 2008 guidelines, recognize that typical bronchoalveolar lavage cellular profiles may allow a diagnosis of sarcoidosis with greater confidence, if the diagnosis is uncertain after clinical assessment and HRCT.
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  • Lymph node biopsy

  • Lung biopsy

Medical management

Management of pulmonary sarcoidosis, asymptomatic disease: as per AAFP 2024 guidelines, do not initiate treatment in patients with no symptoms or mild disease, as spontaneous resolution is common.
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  • Management of pulmonary sarcoidosis (systemic corticosteroids)

  • Management of pulmonary sarcoidosis (ICSs)

  • Management of pulmonary sarcoidosis (methotrexate)

  • Management of pulmonary sarcoidosis (infliximab)

  • Management of pulmonary sarcoidosis (lung transplantation)

  • Management of cardiac sarcoidosis (corticosteroids)

  • Management of cardiac sarcoidosis (pacing)

  • Management of cardiac sarcoidosis (imaging follow-up)

  • Management of cutaneous sarcoidosis

  • Management of neurosarcoidosis

  • Management of sarcoidosis-related fatigue

Preventative measures

Routine immunizations: as per ACR 2023 guidelines, consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in ≥ 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications.
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  • Prophylaxis for P. jirovecii pneumonia