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In the latest Case Record of the Massachusetts General Hospital, a 39-year-old man with a history of psoriasis was admitted to the hospital because of night sweats and abdominal pain. Imaging studies showed numerous ill-defined, hypoattenuating lesions in the spleen and mediastinal and hilar lymphadenopathy.
Gastrointestinal manifestations of sarcoidosis primarily involve the stomach, and patients can present with abdominal pain, nausea, vomiting, and weight loss. Headaches are sometimes a manifestation of sarcoidosis. Hypoattenuating splenic lesions are consistent with sarcoidosis.
Clinical Pearls
• What conditions are associated with erythema nodosum?
Erythema nodosum has been associated with bacterial infections, viral infections, mycoses, drug use (e.g., antibiotics and oral contraceptives), malignant diseases (e.g., lymphoma, leukemia, and other cancers), and other systemic diseases (e.g., sarcoidosis, enteropathies, Sweet’s syndrome, and Takayasu’s disease). Many diseases are associated with erythema nodosum, and among patients in whom a cause of erythema nodosum is identified, streptococcal infection and sarcoidosis are fairly common diagnoses. However, in most patients, an underlying diagnosis is not made.
• What is Lofgren’s syndrome?
The combination of erythema nodosum and ankle pain should raise the possibility of a diagnosis of Lofgren’s syndrome, although the presence of hilar lymphadenopathy is generally also required for this diagnosis. Lofgren’s syndrome is a form of sarcoidosis that is usually self-limited, although its manifestations may persist for several years. A small number of patients with Lofgren’s syndrome have normal chest radiographs at the time of diagnosis; most patients have hilar lymphadenopathy, 13% of patients have respiratory symptoms, and 4% of patients have peripheral lymphadenopathy. Furthermore, 6% of patients have a recurrence of sarcoidosis within 18 months to 20 years after presenting with Lofgren’s syndrome.
Morning Report Questions
Q: What are the typical findings on chest radiography in patients with sarcoidosis?
A: Approximately 85% of patients with sarcoidosis have intrathoracic lymphadenopathy and typically present with bilateral hilar and right paratracheal lymphadenopathy. A predominance of findings in the upper lung with coexistent mediastinal lymphadenopathy are features that suggest sarcoidosis. A perivascular distribution of irregular thickening and small nodules are also characteristic of sarcoidosis.
Q: How does an ACE (angiotensin converting enzyme) level correlate with the likelihood of a diagnosis of sarcoidosis?
A: When the ACE level is less than 25 U per liter, the likelihood ratio for sarcoidosis is 0.12; when the ACE level is between 25 and 71 U per liter, the likelihood ratio is 1.31; and when the ACE level is greater than 71 U per liter, the likelihood ratio increases to 7.15.
