Uveitis, salivary gland swelling, and facial nerve palsy in a febrile woman

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					    CASE REPORT

Uveitis, salivary gland swelling, and
facial nerve palsy in a febrile woman
Bilateral swollen parotid glands trigger an in-depth evaluation and imaging studies that
uncover a rare condition involving ocular, neurologic, and pulmonary abnormalities.

 Jason Evanchan, DO; Timothy J. Barreiro, DO, FCCP,
 FACOI; David Gemmel, PhD

 A 39-year-old woman was evaluated by her primary care
 physician for painless bilateral parotid gland enlargement,
 cough, and dyspnea. She was given a diagnosis of sialo-
 lithiasis and prescribed empiric antibiotics, which yielded
 no improvement. CT of the head and neck revealed parotid
 gland enlargement but no stones or masses. Her chest
 radiograph showed hilar adenopathy. A chest CT, labora-
 tory analysis, and pulmonary consultation were requested.
    Two weeks later, at the pulmonology consult, the patient
 reported an unremarkable past medical history. When
 interviewed, she reported no use of alcohol or tobacco,
 and she had not traveled. Vital signs and oxygenation
 were normal; tympanic temperature was 99°F (37.2°C).
 Physical examination demonstrated bilateral parotid gland
 enlargement and mild weakening of facial muscles, with          FIGURE 1. CT of the head revealed enlargement of the parotid
 the deficit greater on the left. The remainder of the exami-     glands bilaterally (arrows) with no parotid stones.
 nation was unremarkable.
    Diagnostic studies Laboratory analysis revealed a se-
 rum calcium level of 10 mg/dL and a serum angiotensin-
 converting enzyme (ACE) of 65 U/L, both of which were
 at the upper limit of normal. Values for electrolyte, BUN,
 and creatinine, results for liver function tests, and a hemo-
 gram were unremarkable. Pulmonary function test results,
 including diffusing capacity, were normal.
    CT of the nuchal soft tissues revealed asymmetric enlarge-
 ment of the parotid glands with no parotid stones (Figure 1).
 Chest radiograph and CT showed mild adenopathy. Gallium
 GA 67 citrate planar imaging demonstrated bilateral radio-
 nuclide uptake in the lacrimal and parotid glands (Figure 2).
 Bronchoscopy with transbronchial biopsies revealed nonca-
 seating granulomas (Figure 3); results of staining for fungal
 elements and acid-fast bacilli were negative.
    Diagnosis Clinical, laboratory, and histologic findings
 were consistent with sarcoidosis. Findings from a slit-         FIGURE 2. Gallium GA 67 citrate planar image shows the
 lamp examination to rule out optic nerve involvement            panda sign in a patient with sarcoidosis. Note the normal
 were normal. However, a small, elevated, discrete infiltra-      nasopharyngeal uptake. Bilateral radionuclide uptake is seen in
 tion patch that was observed at the choroidal level was         the lacrimal and parotid glands (arrow).

46 JAAPA • MAY 2010 • 23(5) • www.jaapa.com
    CASE REPORT | Heerfordt’s syndrome

                                                                          Frederick Heerfordt, who observed three patients with
                                                                          chronic fevers, enlarged parotid glands, and uveitis. Two of
                                                                          these patients exhibited facial nerve palsy.3,4 Initially attrib-
                                                                          uted to the mumps, uveoparotid fever was recognized as a
                                                                          distinct manifestation of sarcoidosis in 1937.5
                                                                             Etiology The cause of Heerfordt’s syndrome is unclear.
                                                                          Genetic polymorphisms are associated with increased risk
                                                                          of Heerfordt’s syndrome and affect disease presentation.6-8
                                                                          Variations in major histocompatibility and cytokines, such as
                                                                          tumor necrosis factor (TNF), may play a role.9 Fifteen percent
                                                                          of patients with known Heerfordt’s syndrome have a first- or
                                                                          second-degree relative who also has the disorder.6 However,
                                                                          genetics alone does not explain the etiology of the condi-
                                                                          tion. Given the preponderance of pulmonary sarcoidosis, the
                                                                          combined prese
Description: Uveitis can be clinically silent, as was the case with our patient. [...] all patients with sarcoidosis require an ophthalmic examination to evaluate and prevent chronic and possibly permanent ocular damage. While Heerfordt's and Lfgren's syndromes exhibit spontaneous remission, other forms of sarcoidosis can have progressive courses. Because of possible chronicity, patients require lifelong monitoring and follow-up.
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