🧠 TLDR Primary Care - Test your Knowledge (#076)

In this issue:

🫁 Save the Sarcoidosis

✅ Review the common diagnostic guidelines

📚 Key pearls for important patient questions

🧵 And more!

TEST YOUR KNOWLEDGE

Save the Sarcoidosis

Keep sharpening your clinical skills

A 42-year-old female presents with bilateral hilar lymphadenopathy and erythema nodosum. She reports occasional joint pain and shortness of breath. Further investigation reveals elevated levels of angiotensin-converting enzyme (ACE) and non-caseating granulomas on biopsy. A diagnosis of sarcoidosis is suspected. Which of the following is not a correct statement?

[A] Baseline 12-lead electrocardiography should be performed 

[B] Treatment of sarcoidosis is indicated for all cases

[C] Corticosteroids are the first line medication

[D] Methotrexate is the second line medication

Scroll down to find the answer at the end! 👇

Need to refresh your memory before answering this question? Head over to Pathway to review the latest guidelines on Sarcoidosis, as well as some landmark trials. 

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 Society of Cardiology (ESC 2023; 2021), among others.

Case Conclusion

Keep your clinical skills sharp

Answer - B. Treatment of sarcoidosis is indicated for all cases

Explanation - This patient’s presentation is consistent with Sarcoidosis, a multisystem granulomatous inflammatory disease that can present at any age and with any organ involvement. Choice B is wrong here due to sarcoidosis having a chance of spontaneous resolution in asymptomatic or mild cases. These cases do not require treatment. 

Treatment is indicated in symptomatic pulmonary sarcoidosis. Corticosteroids are recommended as the first-line therapy, with methotrexate being considered a second-line therapy. However, there is limited evidence that corticosteroids improve lung function in the long term. An initial prednisone dosage of 0.5 to 1.0 mg per kg per day (usually 20 to 40 mg per day) can be considered per the most recent AAFP guidelines. In patients without cardiac symptoms, a baseline EKG should be obtained to screen for cardiac involvement. 

What are the guideline recommendations for the diagnosis of sarcoidosis?

• Consider obtaining a complete blood cell count in patients with sarcoidosis, in order to screen for hematological abnormalities. (C)
• Obtain baseline serum calcium in patients with sarcoidosis, in order to screen for abnormal calcium metabolism. (B)
• Consider measuring both 25-OH vitamin D and 1, 25-OH vitamin D levels in patients with sarcoidosis in whom assessment of vitamin D metabolism is deemed necessary, such as to determine if vitamin D replacement is indicated. (C)
• Obtain baseline 12-lead ECG in patients with extracardiac sarcoidosis without cardiac symptoms to screen for cardiac involvement. (B)
• Consider obtaining a baseline eye examination in patients with sarcoidosis (even in the absence of ocular symptoms), in order to screen for ocular sarcoidosis. (C)
• When diagnosing sarcoidosis, a holistic approach should be implemented that focuses on clinical presentation and radiographic findings, biopsy with evidence of noncaseating granulomas, involvement of more than one organ system, and the exclusion of other etiologies of granulomatous disease. (C)
• Recognize that typical BAL cellular profiles may allow a diagnosis of sarcoidosis with greater confidence if the diagnosis is uncertain after clinical assessment and HRCT. (B)
• Recognize that serum angiotensin-converting enzyme level has only a limited role in diagnosis and does not contribute to monitoring patients with pulmonary sarcoidosis when added to serial lung function and imaging. (B)
• A tissue biopsy of cutaneous lesions or peripheral lymph nodes is the least invasive and safest option for diagnosis. (E)
• Histopathologic findings of sarcoidosis are characterized by noncaseating granulomas consisting of aggregates of epithelioid histiocytes, giant cells, and mature macrophages. (E)

What are the guideline recommendations for the treatment of sarcoidosis?

• Do not initiate treatment in patients with no symptoms or mild disease, as spontaneous resolution is common. (D)
• Initiate corticosteroids as first-line therapy in patients with pulmonary sarcoidosis. (B)
• Administer prednisolone (or equivalent) 0.5 mg/kg/day for 4 weeks, then reduce to a maintenance dose to control symptoms and disease progression for a period of 6-24 months. (B)
• Do not use ICSs routinely for the treatment of chronic cough in patients with pulmonary sarcoidosis. (D)
• Reserve methotrexate as second-line therapy for high-risk patients with continued disease despite corticosteroids or patients unable to tolerate ongoing corticosteroid therapy. (B)
• Consider initiating TNF-α inhibitors in patients with sarcoidosis not responding to traditional therapies. (C)

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