MKSAP Quiz: Abnormal finding on a chest radiograph

MKSAP Answer and Critique

The correct answer is D. Observation. This content is available to MKSAP subscribers as Question 16 in the Pulmonary and Critical Care Medicine section. More information about MKSAP is available online.

The most appropriate management for this patient is observation (Option D). The patient most likely has stage I pulmonary sarcoidosis, a granulomatous disease of unknown cause that can affect any organ system but most commonly affects the lungs. Many patients are asymptomatic, and lung involvement is found incidentally on a chest radiograph obtained for other reasons. Pulmonary sarcoidosis is classified into four stages based on appearance on chest radiograph: stage I, bilateral hilar lymphadenopathy; stage II, bilateral hilar lymphadenopathy with pulmonary infiltrates; stage III, parenchymal infiltrates alone; and stage IV, pulmonary fibrosis with parenchymal distortion or bullae. For patients with stage I disease, clinical diagnosis based on a classic presentation is a cost-effective strategy, with pretest probability approaching 99.95%. The overall prognosis of stage I pulmonary sarcoidosis is excellent, with spontaneous regression of radiographic abnormalities observed in most patients.

Diagnosis of pulmonary sarcoidosis, with a few exceptions, typically requires bronchoscopic biopsy (Option A), with tissue obtained from a lymph node or from the pulmonary parenchyma. The diagnosis is made by the finding of noncaseating granulomas with exclusion of potential mimicking infections (mycobacteria, fungi), exclusion of other systemic granulomatous diseases, and involvement of more than one organ system. The absence of respiratory and constitutional symptoms makes infectious and malignant causes unlikely. Clinical presentations of sarcoidosis that do not require a biopsy include asymptomatic stage I pulmonary sarcoidosis (absence of fevers, malaise, or night sweats to suggest a malignancy); Löfgren syndrome (bilateral hilar lymphadenopathy, migratory polyarthralgia, erythema nodosum, and fever); and Heerfordt syndrome (anterior uveitis, parotiditis, fever, and facial nerve palsy). This asymptomatic patient with stage I pulmonary sarcoidosis does not need a bronchoscopic biopsy.

The primary treatment for symptomatic pulmonary sarcoidosis is systemic glucocorticoids. Methotrexate (Option B) may be used for patients who cannot tolerate glucocorticoids due to adverse effects or those who continue to have disabling symptoms despite glucocorticoid treatment. Methotrexate may also be used as a glucocorticoid-sparing therapy to limit adverse effects from long-term use of glucocorticoids. Neither glucocorticoids nor methotrexate is indicated for asymptomatic stage I pulmonary sarcoidosis.

Glucocorticoids, typically prednisone (Option C), are the mainstay of therapy for sarcoidosis. However, initial treatment is usually limited to symptomatic patients with pulmonary/extrapulmonary organ dysfunction. Patients who require long-term glucocorticoids are frequently transitioned to glucocorticoid-sparing agents such as methotrexate or leflunomide. Treatment with prednisone would not be the most appropriate initial treatment for this asymptomatic patient with stage I disease.

Key Points

• Clinical presentations of sarcoidosis that do not require a biopsy include asymptomatic stage I pulmonary sarcoidosis, Löfgren syndrome, and Heerfordt syndrome.
• The overall prognosis of stage I pulmonary sarcoidosis is excellent, with spontaneous regression of radiographic abnormalities observed in most patients.