A Clinical Case Worthy of Sherlock Holmes
A 35-year-old man was transferred to our emergency department for evaluation of chest pain and shortness of breath of 1 week's duration. The patient's medical history was significant for a month-long episode of bilateral ankle inflammation at 8 years of age following a viral syndrome. He was treated at that time with nonsteroidal anti-inflammatory drugs. Blood tests showed a slightly elevated white blood cell count of 12,500 cells/µL, a platelet count of 480,000 cells/µL, erythrocyte sedimentation rate (ESR) of 77 mm/h, and C-reactive protein (CRP) level of 6 mg/L. Antinuclear antibody (ANA), rheumatoid factor, and antistreptolysin O titers were normal.
The patient was then well until 24 years of age when, after an upper respiratory tract illness, he presented with an episode of shortness of breath and chest pain on breathing and lying flat, along with bilateral knee, wrist, and ankle inflammation. Echocardiography revealed pericardial thickening and fluid without tamponade physiology. On blood tests, he had an elevated white blood cell count of 22,000 cells/µL, platelet count of 600,500 cells/µL, ESR of 88 mm/h, and CRP level of 10 mg/L. Lupus and rheumatoid arthritis serologies were normal, the serum ferritin level was 18,000 ng/mL, and viral and streptococcal tests were normal. The patient responded to a course of prednisone, initially 40 mg/day and then tapered off over 2 weeks.
Two weeks ago, the patient developed coryza, nonproductive cough, and low-grade fever and was treated by his primary care physician with azithromycin without improvement. One week ago, he presented to a local emergency department with severe anterior pleuritic pain and shortness of breath; temperature of 39.6º C, occurring mainly in the evening; bilateral wrist and ankle inflammation; and fatigue. His white blood count was 38,000 cells/µL, platelet count 650,000 cells/µL, ESR 100 mm/h, CRP level 15 mg/L, and hemoglobin level 9 g/dL.
Samples for culture were obtained, and the patient was treated with broad-spectrum antibiotics, with no improvement in any of his symptoms. He was then transferred to our hospital for further assessment and treatment.
Hematology/oncology and infectious disease consults were requested; aside from splenomegaly on abdominal CT and increased myeloid elements seen on bone marrow, the evaluation was negative. A rheumatology consult was requested because of the patient's medical history and the possibility of adult-onset Still disease.
All autoimmune serologic tests were normal, including ANA, rheumatoid factor, anti-cyclic citrullinated peptide, and perinuclear and cytoplasmic antineutrophil cytoplasmic antibody. His C3 and C4 complement levels were elevated, and the serum ferritin level was 82,000 mg/mL.
Echocardiography showed both a moderate-sized pericardial effusion and a wall-motion abnormality, suggesting myocardial dysfunction. Electrocardiography showed diffuse ST and T-wave changes, with a mild elevation in troponin. An endomyocardial biopsy was performed and showed myocarditis with mixed cellularity, without giant cells, amyloid, iron, or eosinophils.
Clinical Presentation
A 35-year-old man was transferred to our emergency department for evaluation of chest pain and shortness of breath of 1 week's duration. The patient's medical history was significant for a month-long episode of bilateral ankle inflammation at 8 years of age following a viral syndrome. He was treated at that time with nonsteroidal anti-inflammatory drugs. Blood tests showed a slightly elevated white blood cell count of 12,500 cells/µL, a platelet count of 480,000 cells/µL, erythrocyte sedimentation rate (ESR) of 77 mm/h, and C-reactive protein (CRP) level of 6 mg/L. Antinuclear antibody (ANA), rheumatoid factor, and antistreptolysin O titers were normal.
The patient was then well until 24 years of age when, after an upper respiratory tract illness, he presented with an episode of shortness of breath and chest pain on breathing and lying flat, along with bilateral knee, wrist, and ankle inflammation. Echocardiography revealed pericardial thickening and fluid without tamponade physiology. On blood tests, he had an elevated white blood cell count of 22,000 cells/µL, platelet count of 600,500 cells/µL, ESR of 88 mm/h, and CRP level of 10 mg/L. Lupus and rheumatoid arthritis serologies were normal, the serum ferritin level was 18,000 ng/mL, and viral and streptococcal tests were normal. The patient responded to a course of prednisone, initially 40 mg/day and then tapered off over 2 weeks.
Two weeks ago, the patient developed coryza, nonproductive cough, and low-grade fever and was treated by his primary care physician with azithromycin without improvement. One week ago, he presented to a local emergency department with severe anterior pleuritic pain and shortness of breath; temperature of 39.6º C, occurring mainly in the evening; bilateral wrist and ankle inflammation; and fatigue. His white blood count was 38,000 cells/µL, platelet count 650,000 cells/µL, ESR 100 mm/h, CRP level 15 mg/L, and hemoglobin level 9 g/dL.
Samples for culture were obtained, and the patient was treated with broad-spectrum antibiotics, with no improvement in any of his symptoms. He was then transferred to our hospital for further assessment and treatment.
Hematology/oncology and infectious disease consults were requested; aside from splenomegaly on abdominal CT and increased myeloid elements seen on bone marrow, the evaluation was negative. A rheumatology consult was requested because of the patient's medical history and the possibility of adult-onset Still disease.
All autoimmune serologic tests were normal, including ANA, rheumatoid factor, anti-cyclic citrullinated peptide, and perinuclear and cytoplasmic antineutrophil cytoplasmic antibody. His C3 and C4 complement levels were elevated, and the serum ferritin level was 82,000 mg/mL.
Echocardiography showed both a moderate-sized pericardial effusion and a wall-motion abnormality, suggesting myocardial dysfunction. Electrocardiography showed diffuse ST and T-wave changes, with a mild elevation in troponin. An endomyocardial biopsy was performed and showed myocarditis with mixed cellularity, without giant cells, amyloid, iron, or eosinophils.
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