Sarcoidosis of the lung
Patient Presentation:
A 28-year-old African American female (HM) with no significant past medical history presents to the office complaining of shortness of breath beginning suddenly three days ago while she was sitting at work as a librarian at the local county library. Patient describes shortness of breath as “pressure and tightness” in upper chest area that is constant and is unaffected by physical activity and/or rest. Patient also reports a dry cough, mild chest pain localized to the same location of as the pressure, and feelings of weakness, malaise, and fever (denies using thermometer to measure temperature). Patient reports trying to “rest” by not going to work for two days in order to relieve dyspnea; however, patient denies feeling any relief of symptoms since onset three days ago hence her presence at the office today. Patient reports severity as 6 out of 10; however, she complains of increasing anxiety due to prolonged presence of symptoms. Of note, patient denies history of hemoptysis, sleep apnea, asthma, bronchitis, TB, recent travel, sick contact and occupational or environmental exposure to toxins. Patient reports up-to-date immunizations and denies medication and/or environmental allergies. Physical examination (PE) is significant for normal vital signs except a fever noted at 101 degrees F. PE also revealed normal thorax with no apparent deformities or trauma with normal respiratory movements (no retractions or use of accessory muscles for breathing). On auscultation, dry frequent cough noted and wheezes heard bilaterally in upper lobes; lower lobes are clear and equal bilaterally. Heart sounds normal S1/S2 with no audible murmurs or rubs.
Differential List:
Given the non-emergent nature of the patient’s condition, she was worked up for non-emergent dyspnea with the initial assumption that the causative agent was infectious, such as bronchitis, pneumonia or tuberculosis. However, as soon as the radiological findings and lab work were complete, the clinical picture became clearer and the patient was diagnosed with sarcoidosis of the lung. The definitive findings that confirmed this diagnosis were bilateral hilar and mediastinal lymphadenopathy, pulmonary opacities and bronchial wall thickening. Chest radiograph revealed Stage II Sarcoidosis of the lung, which consists mostly of bilateral hilar adenopathy and reticular opacities in the upper lung zones. Patients with stage II sarcoidosis typically present with mild to moderate symptoms, most commonly cough, dyspnea, fever and/or easy fatigue.
Diagnosis:
Lab work revealed: eosinophilia, high erythrocyte sedimentation rate, negative for TB exposure or infection and hypergammaglobulinemia
Pulmonary function test revealed: Normal lung function. This is a normal finding in patients with mild to moderate, spontaneously occurring sarcoidosis of the lung. Pulmonary function tests are used to assess the course of the disease in the individual patient by sequential measurement. Bronchoalveolar lavage revealed: reduced number of CD8 cells, elevated CD4 to CD8 ratio and an increased amount of activated T cells, CD4 cells, immunoglobulins and IgG-secreting cells. Bronchoscopy with biopsy revealed noncaseating granulomas, which is the characteristic histopathological abnormality seen in sarcoidosis. Chest radiograph and CT scan revealed: Stage II sarcoidosis with bilateral hilar and mediastinal lymphadenopathy, reticular opacities in upper lung zones and bronchial wall thickening.
Treatment:
In most cases of mild sarcoidosis of the lung, symptoms resolve on their own without intervention or treatment; however, given that the patient reports increased anxiety and inability to fulfill activities of daily living, patient is prescribed oral glucocorticoids (prednisone typically) with an increased initial therapeutic dose, which decreases gradually into a maintenance dose. The glucocorticoids are prescribed to reduce the burden of the granulomatous inflammation and prevent the development of irreversible end-organ damage. Most patients discontinue glucocorticoids within one year. Follow-up every 4-8 weeks is required to monitor glucocorticoid use and side effects, as well as monitor disease course by assessing pulmonary function tests, spirometry, diffusing capacity, ambulatory oximetry, relevant lab work and chest x-rays. ROS and PE should also be completed at least every 3 months while disease is still active. In rare cases, condition may worsen or become chronic in which case lung transplant is considered. Care after lung transplant would require intense immunosuppressant therapy to prevent both rejection and sarcoidosis of the new lung tissue.
Outcome:
In most cases, patients recover from active disease within two years of onset of symptoms without any long-term complications or end-organ damage. In this patient’s case, long-term implications of glucocorticoid use must be assessed and treatment cessation will require medication tapering and frequent follow-up.
Case created by Hannah MacIntyre, 2011.