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    54-year-old African American woman has hypercalcemia and an elevated creatinine level

    A 54-year-old African American woman who just moved to the area has hypercalcemia and an elevated creatinine level on routine laboratory testing. After you review the patient's medical records from her previous physician, you note that she has had hypercalcemia for nearly 1 year. She denies any complaints except for an unintentional weight loss of 20 pounds in the last few months. There are no other laboratory abnormalities except for proteinuria.

    Findings on chest x-ray show diffuse interstitial lung disease, and computed tomography of her chest shows interstitial thickening with perilymphatic micronodules and subpleural reticulation, mostly in the bilateral upper lobes. Bronchoscopy with transbronchial biopsy is performed. Cultures and stains are negative. Pathology shows noncaseating granulomas.

    What is the most appropriate management at this time?

    • isoniazid and pyridoxine
    • referral for chemotherapy
    • continued observation
    • isoniazid, rifampin, pyrazinamide, and ethambutol
    • oral corticosteroid therapy
    The correct answer is:

    oral corticosteroid therapy

     

    Educational Objective:

    Characterize how to diagnose and manage ILD.

    Explanation:

    The most likely diagnosis in this patient is sarcoidosis, which is a systemic inflammatory disease with pathology typified by noncaseating granulomas. Any organ system can be involved, but more than 90% of those with sarcoidosis have pulmonary involvement at the time of diagnosis; chest abnormalities on x-ray can also be seen in more than 90% of these patients.

    There are 5 stages of chest involvement evidenced on x-ray:

    • Stage 0: Normal
    • Stage 1: Bilateral hilar lymphadenopathy
    • Stage 2: Bilateral hilar lymphadenopathy, parenchymal infiltrates (reticular opacities)
    • Stage 3: Parenchymal infiltrates, no bilateral hilar lymphadenopathy (shrinking nodes)
    • Stage 4: Reticular opacities with evidence of volume loss (predominantly mid- and upper-lung zones)

    Masses, traction bronchiectasis, calcification, cyst/bullae formation, and disease progression can lead to distortion or destruction of the lung architecture.

    Computed tomography of the chest is not usually needed for diagnosis, but typical findings include micronodules along the bronchovascular bundles with upper-lobe predominance. An elevated level of angiotensin-converting enzyme is seen in 30% to 80% of patients, but this finding is nonspecific, as are other laboratory findings such as hypercalcemia (1%-4%), hypercalciuria (15%-40%), and polyclonal hypergammaglobulinemia (30%-80%).

    Treatment is indicated when severe organ dysfunction, including pulmonary symptoms, and myocardial, central nervous system, or ophthalmologic complications are present. Chronic hypercalcemia is also an indication for treatment. Corticosteroids are usually the first line of treatment. If the patient responds favorably, then corticosteroids can be tapered after 3 months.

    Reference:

    Pereira CA, Dornfeld MC, Baughman R, et al. Clinical phenotypes in sarcoidosis. Curr Opin Pulm Med. 2014;20:496-502.

     

    This question appears in Med-Challenger Physician Assistant Exam Review with CME

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