Sarcoidosis

Sarcoidosis is a multisystem non-caseating granulomatous disease that often hides behind common emergency department complaints. Bilateral hilar adenopathy, lupus pernio, hypercalcemia, and heart block are the high-yield clues, while chronic steroids, cytotoxic agents, and biologics can reshape both presentation and ED treatment.

Recognizing Sarcoidosis in the ED

• Multisystem granulomatous pattern: Sarcoidosis causes non-caseating granulomas that can scar over time, so patients may look well early and later present with pulmonary, ocular, lymphatic, musculoskeletal, or constitutional symptoms.
• Pulmonary involvement dominance: Pulmonary disease shows up in about 90% of patients, with dyspnea and cough as the usual complaints and bilateral hilar adenopathy as the classic chest x-ray clue.
• Lupus pernio finding: Lupus pernio is the most specific skin finding: violaceous, shiny nodules on the cheeks, neck, or ears that should immediately raise concern for sarcoidosis.
• Diagnosis needs tissue: Chest x-ray may be the first hint, but biopsy is needed to confirm sarcoidosis and exclude mimics such as lymphoma. We get into the mimics worth keeping on your radar in the episode.
• Who gets complicated disease: In the US, sarcoidosis disproportionately affects African Americans and women, with symptom onset usually between ages 20 and 40 and a higher burden of complications in African American patients.

Emergency Complications and Management Pearls

• Cardiac sarcoid red flags: Chest pain or syncope in sarcoidosis should trigger concern for myocarditis, restrictive cardiomyopathy, ventricular irritability, and heart block from granulomas disrupting conduction tissue.
• Neurosarcoid presentations: Neurosarcoidosis can produce aseptic meningitis, hydrocephalus, seizures, psychosis, or cranial nerve palsies, especially VII, IX, and X, making CT brain and metabolic evaluation reasonable first steps.
• Hypercalcemia mechanism: Macrophage-driven vitamin D activation can cause hypercalcemia and near-universal hypercalciuria, so stones, abdominal complaints, and psychiatric changes may be the sarcoid clue rather than the diagnosis itself.
• Medication history matters: Chronic prednisone, methotrexate, azathioprine, mycophenolate, cyclophosphamide, or adalimumab can create immunosuppression, cytopenias, GI toxicity, and drug-interaction problems that change ED decisions.
• Thrombosis and disposition risk: Sarcoidosis carries a roughly three-fold increased VTE risk, and once ocular, neurologic, cardiac, or hypercalcemic disease appears, many patients need prolonged therapy and a low threshold for admission. We walk through the ED mindset for that disposition call in the chapter.

References:

1. Ackermann D. Die Hyperkalzämie im Verlauf der Sarkoidose--Fallbeispiel, Prävalenz, Pathophysiologie und Therapiemöglichkeiten [Hypercalcemia in sarcoidosis--case report, prevalence, pathophysiology and therapeutic options]. Ther Umsch. 2007;64(5):281-286. PMID: 17685088.
2. Ramy Yakobi MD. Acute complications of sarcoidosis: Practice Essentials, patient history, physical examination. Acute Complications of Sarcoidosis: Practice Essentials, Patient History, Physical Examination. https://emedicine.medscape.com/article/809047-overview#showall. Published March 15, 2022. Accessed September 26, 2022.
3. Redissi A, et al. Lupus Pernio. In: StatPearls. Treasure Island (FL): StatPearls Publishing; February 20, 2023. PMID: 30725653
4. Sarcoidosis. WikEM. https://wikem.org/wiki/Sarcoidosis. Published February 1, 2021. Accessed September 26, 2022.
5. Simon E. Approach to patient with sarcoidosis in the ED setting. emDOCs.net - Emergency Medicine Education. Published July 20, 2017. Accessed September 26, 2022. 
6. Ungprasert P, Crowson CS, Matteson EL. Association of Sarcoidosis With Increased Risk of VTE: A Population-Based Study, 1976 to 2013. Chest. 2017;151(2):425-430. PMID: 27687848