A Man with Sore Throat and Myalgias

Posted by • July 17th, 2015

A Man with Sore Throat and Myalgias 7-17-15In the latest Case Record of the Massachusetts General Hospital, a 20-year-old man presented with fever and a pericardial effusion. Five weeks earlier, sore throat, fever, malaise, and myalgias had developed. Broad-spectrum antibiotic therapy was administered, without improvement. A diagnosis was made.

A number of case reports indicate an association between tamponade and adult-onset Still’s disease. One case report describes a patient with adult-onset Still’s disease and reversible constrictive pericarditis.

Clinical Pearls

What clinical and laboratory findings are associated with adult-onset Still’s disease?

Adult-onset Still’s disease is classically characterized by four cardinal symptoms: spiking fever, evanescent salmon-pink maculopapular rash, arthritis, and a white-cell count greater than 10,000 per cubic millimeter, with predominance of neutrophilic polymorphonuclear cells. The rash associated with adult-onset Still’s disease is nonpruritic; it occurs with fever and commonly involves the trunk, arms, and legs. Persistent, atypical rashes may also occur. The arthralgias and arthritis associated with adult-onset Still’s disease generally involve the larger joints (i.e., knees, wrists, elbows, ankles, and shoulders). In addition, many affected patients present with sore throat, lymphadenopathy, anemia, and abnormal results of liver-function tests, and approximately one quarter of patients have pleuritis or pericarditis. Most patients have markedly elevated ferritin levels. Pericardial effusion occurs in approximately 4% of patients with adult-onset Still’s disease, and pleural effusion occurs in approximately 18%.

Figure 1. Clinical Photograph.

Does adult-onset Still’s disease have more than one clinical phenotype?

Adult-onset Still’s disease has two clinical phenotypes — a systemic form, which is predominantly characterized by systemic symptoms (e.g., high fever and rash), and a chronic form, which is predominantly characterized by arthritis that may become deforming. The systemic form is associated with a better prognosis and may be monophasic.

Morning Report Questions

Q: Are there diagnostic laboratory tests or specific histologic findings for adult-onset Still’s disease?

A: In patients with adult-onset Still’s disease, synovial or pleuropericardial fluids are sterile, inflammatory exudates. In addition, skin-biopsy or lymph-node-biopsy specimens have no specific histologic appearance. A low fraction of glycosylated ferritin may have specificity for adult-onset Still’s disease, but a test for this is not widely available. No laboratory tests are specific for adult-onset Still’s disease, and therefore the diagnosis of this disorder relies on the presence of a constellation of clinical findings. Because adult-onset Still’s disease remains a diagnosis of exclusion, there is often a considerable delay in establishing the diagnosis. Differentiating between adult-onset Still’s disease and acute rheumatic fever has long been a clinical challenge because of their overlapping clinical features. Patients with adult-onset Still’s disease may have a higher maximum temperature and greater temperature swings than do patients with acute rheumatic fever. The Yamaguchi criteria are the most validated diagnostic criteria for adult-onset Still’s disease, although the Yamaguchi criteria are considered valid only in the absence of infection.

Table 3. Diagnostic Criteria for Adult-Onset Still’s Disease and Acute Rheumatic Fever.

Q: How is adult-onset Still’s disease treated?

A: Adult-onset Still’s disease is characterized by inflammation, and interleukin-1 appears to be the dominant mediator. Biologic agents that block interleukin-1 activity are highly effective in the treatment of persons with adult-onset Still’s disease. However, evidence for the treatment of adult-onset Still’s disease is based on small studies and case series. For mild disease, glucocorticoids and nonsteroidal antiinflammatory drugs may be effective. For moderate disease, glucocorticoids are typically combined with methotrexate for chronic forms or combined with biologically active agents for systemic forms. For severe disease, evidence is limited.

2 Responses to “A Man with Sore Throat and Myalgias”

  1. GAIal abo frag says:

    Wonder full

  2. Chien-Wei Wu says:

    It’s a very interesting case to me. I’m a intern doctor in Taipei, Taiwan. In our hospital, we have not seen this king of case before. Especially, the chief complaint is sore throat and myalgias, I will make a diagnosis of acute URI or flu.
    Thanks to the editor for sharing this case.