What is the best next step in managing a 36-year-old woman with fever, dyspnea, cough, and fatigue, hypercalcemia, and bilateral hilar lymph node enlargement with upper lobe reticular opacities on chest imaging?

From the Guidelines

The best next step in managing this 36-year-old woman is to perform a transbronchial biopsy, as this will provide a definitive diagnosis and guide further treatment. This patient's presentation strongly suggests sarcoidosis, with the classic triad of bilateral hilar lymphadenopathy, pulmonary infiltrates, and hypercalcemia, along with constitutional symptoms of fever, dyspnea, cough, and fatigue 1. However, a definitive diagnosis requires histopathological confirmation of non-caseating granulomas and exclusion of other granulomatous diseases.

According to the American Thoracic Society clinical practice guideline, the diagnosis of sarcoidosis is supported by clinical features such as bilateral hilar adenopathy, perilymphatic nodules, and hypercalcemia or hypercalciuria with abnormal vitamin D metabolism 1. The guideline also recommends that asymptomatic bilateral hilar lymphadenopathy confirmed to have sarcoidosis does not require sampling lymph nodes, but this patient is symptomatic, making a tissue biopsy necessary.

Some key points to consider in the diagnosis and management of sarcoidosis include:

• The presence of non-caseating granulomas on histopathology is essential for diagnosis
• The disease can affect multiple organs, including the lungs, skin, eyes, and heart
• Treatment typically involves oral corticosteroids, with the goal of suppressing granulomatous inflammation and improving symptoms
• The decision to treat depends on disease severity, organ involvement, and symptom burden, as some cases of sarcoidosis resolve spontaneously 1.

In this case, given the patient's symptoms and radiographic findings, a transbronchial biopsy is the best next step to confirm the diagnosis and guide further management.

From the Research

Diagnosis and Management of Sarcoidosis

The patient's symptoms, including fever, dyspnea, cough, and fatigue, along with hypercalcemia and bilateral hilar lymph node enlargement with upper lobe reticular opacities on chest imaging, suggest a diagnosis of sarcoidosis.

Diagnostic Options

• Measurement of ACE level: While angiotensin-converting enzyme (ACE) levels can be elevated in sarcoidosis, this test is not specific and can be normal in many patients with the disease 2.
• FDG-PET scan: This imaging modality can be useful in detecting occult sites of disease and guiding biopsy to active sites, especially when combined with CT scan 2, 3, 4, 5.
• CT angiography: This test is not typically used in the diagnosis of sarcoidosis.
• Transbronchial biopsy: This procedure can provide histological confirmation of sarcoidosis by demonstrating noncaseating granulomas, and is considered a key diagnostic tool in the disease 2, 6.

Best Next Step

Given the patient's presentation and the need for a definitive diagnosis, the best next step would be to perform a transbronchial biopsy to obtain tissue for histological examination. This procedure can provide a definitive diagnosis of sarcoidosis by demonstrating noncaseating granulomas. Additionally, an FDG-PET scan could be considered to identify occult sites of disease and guide biopsy to active sites.

Key Considerations

• The diagnosis of sarcoidosis relies on a combination of clinical, radiological, and histological findings.
• Transbronchial biopsy is a key diagnostic tool in sarcoidosis, with a high sensitivity for detecting noncaseating granulomas 6.
• FDG-PET scan can be useful in detecting occult sites of disease and guiding biopsy to active sites, especially when combined with CT scan 2, 3, 4, 5.