A 52-year-old man comes to the physician for intermittent sore throat, nocturnal high fevers, cold sweats, fatigue, anorexia, and weight loss over the last 4 weeks. He also complains of pain and swelling of his hands and wrists and brief episodes of a rash. He does not recall any tick bites and has no travel history or ill contacts. His temperature is 39 C (102.2 F), blood pressure is 100/70 mm Hg, and pulse is 92/min. Oropharyngeal mucosa is dry without exudates. There are several enlarged cervical lymph nodes. A scarce macular, nontender, purple rash is noted on both arms. There are no heart murmurs. Splenomegaly is noted. Synovitis is present in several joints including both hands, wrists, and elbows. A moderate effusion is present in the left knee joint. Laboratory results are as follows:

Which of the following is the most likely diagnosis?

Question

A 52-year-old man comes to the physician for intermittent sore throat, nocturnal high fevers, cold sweats, fatigue, anorexia, and weight loss over the last 4 weeks. He also complains of pain and swelling of his hands and wrists and brief episodes of a rash. He does not recall any tick bites and has no travel history or ill contacts. His temperature is 39 C (102.2 F) , blood pressure is 100/70 mm Hg, and pulse is 92/min. Oropharyngeal mucosa is dry without exudates. There are several enlarged cervical lymph nodes. A scarce macular, nontender, purple rash is noted on both arms. There are no heart murmurs. Splenomegaly is noted. Synovitis is present in several joints including both hands, wrists, and elbows. A moderate effusion is present in the left knee joint. Laboratory results are as follows:

Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_993c_d143_853d_83a86d308fd9_MD0002_00 Adult-onset Still's disease (AOSD), also known as systemic-onset juvenile idiopathic arthritis, is an inflammatory disorder characterized by high daily recurring fevers, polyarthritis, and neutrophilic leukocytosis. A transient, faint, salmon-colored, macular skin rash characteristically comes and goes and is usually not pruritic. Additional features may include lymphadenopathy, sore throat, hepatosplenomegaly with elevated liver markers, reactive thrombocytosis, normocytic anemia, pleuritis, and pericarditis. However, many of these clinical features may not be present and the diagnosis of AOSD must be made after excluding infections and malignancies. rash AOSD is mediated by high levels of inflammatory cytokines (IL-1, IL-6, TNF-α) and is associated with significant elevations of inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein. The most useful diagnostic test, however, is serum ferritin, which is markedly elevated, often in the thousands. More specific rheumatologic serologies, such as antinuclear antibodies and rheumatoid factor (RF), are negative. Mild cases of AOSD may be managed with nonsteroidal anti-inflammatory drugs, but most patients will require moderate- to high-dose glucocorticoids. (Choice B) Felty syndrome is a variant of rheumatoid arthritis characterized by splenomegaly and leukopenia. It generally occurs in patients with long-established rheumatoid arthritis and most will have a positive RF. (Choice C) Bacteremia with Neisseria meningitidis is characterized by acute onset of fever with headache, stiff neck, and a petechial or hemorrhagic rash. Arthritis, if present, is likely to be mono- rather than poly-arthritic. (Choice D) Post-streptococcal reactive arthritis is a polyarticular inflammatory syndrome occurring 1-2 weeks after group A streptococcal infections. By convention, patients do not meet criteria for acute rheumatic fever and do not appear at risk for carditis. This patient's cyclical fever, leukocytosis, and splenomegaly are more characteristic of AOSD. (Choice E) The rash of Sweet syndrome is tender, erythematous, and consists of well-demarcated papules and plaques; it is not transient, as in this patient. Sweet syndrome Educational objective: Adult-onset Still's disease is characterized by recurrent high fever, polyarticular arthritis, evanescent macular rash, leukocytosis, and elevated serum ferritin levels. Other nonspecific clinical features such as arthritis, sore throat, lymphadenopathy, and elevated liver markers may be seen. __________ References: Clinical features and prognosis in 82 patients with adult-onset Still's disease. (http://www.ncbi.nlm.nih.gov/pubmed/24050706) Ferritin in adult-onset still's disease: just a useful innocent bystander? (http://www.ncbi.nlm.nih.gov/pubmed/22536541) Sweet's syndrome: a revisit for hematologists and oncologists. (http://www.ncbi.nlm.nih.gov/pubmed/23046529)

A 52-Year-Old Man Comes to the Physician for Intermittent Sore