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Adult-onset Still's disease with disseminated intravascular coagulation and hemophagocytic syndrome: a case report.

Mimura T, Shimodaira M, Kibata M, Tsukadaira A, Shirahata K - BMC Res Notes (2014)

Bottom Line: The combination of nafamostat mesilate and prednisolone therapies caused a rapid reduction in the fever and rash.The inflammatory markers decreased immediately, and disseminated intravascular coagulation improved.Her symptoms resolved with low-dose prednisolone treatment, and she was monitored thereafter at our outpatient clinic.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Iida Municipal Hospital, 438 Yawata-machi, Iida, Nagano 395-8502, Japan. masanori19810813@yahoo.co.jp.

ABSTRACT

Background: Adult-onset Still's disease is a rare inflammatory condition of unknown origin characterized by high spiking fever, arthralgia, arthritis, myalgia, salmon-colored evanescent rash, and hepatosplenomegaly. The diagnosis of adult-onset Still's disease requires the exclusion of other possible disorders because it lacks specific clinical and histopathological findings. Adult-onset Still's disease rarely become fatal due to visceral involvements such as disseminated intravascular coagulation.

Case presentation: A 22-year-old Chinese female presented to our medical center with high spiking fever for one week, myalgia for two weeks, and arthralgia and pink maculopapular rash for four weeks. She developed disseminated intravascular coagulation on the fourth day after admission. There was no other explanation for the fever and rash, including infection, malignancy, and collagenosis. Together, the high spiking fever, salmon-colored rash, splenomegaly, and excess hepatic enzyme, indicated adult-onset Still's disease based on the Yamaguchi criteria. Therefore, prednisolone therapy was initiated. The combination of nafamostat mesilate and prednisolone therapies caused a rapid reduction in the fever and rash. The inflammatory markers decreased immediately, and disseminated intravascular coagulation improved. Her symptoms resolved with low-dose prednisolone treatment, and she was monitored thereafter at our outpatient clinic.

Conclusion: The previous use of nonsteroidal anti-inflammatory drugs could have caused disseminated intravascular coagulation in this patient with adult-onset Still's disease. We propose that physicians should consider the possibility of disseminated intravascular coagulation as a complication during the course of adult-onset Still's disease and suggest that prednisolone therapy should be initiated in the early stages of adult-onset Still's disease.

No MeSH data available.


Related in: MedlinePlus

Clinical course of a patient with adult-onset Still’s disease. ALT, alanine transaminase; AST, aspartate transaminase; BT, body temperature; CRP, C-reactive protein; WBC, white blood cell count.
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Fig3: Clinical course of a patient with adult-onset Still’s disease. ALT, alanine transaminase; AST, aspartate transaminase; BT, body temperature; CRP, C-reactive protein; WBC, white blood cell count.

Mentions: After admission, the high fever persisted, with increasing levels of hepatic and biliary enzymes (Figure 3). On day 4 after admission, the platelet count suddenly decreased to 6.3 × 104/μL, and the fibrinogen level decreased to 122 mg/dL (normal: 190–430 mg/dL). In contrast, the patient had excess fibrin degradation product (FDP; 45.5 μg/mL; normal: 1–10 μg/mL), and a high prothrombin time-international normalized ratio (PT-INR: 1.23; control: 1.0). Based on these data, she was diagnosed with DIC and nafamostat mesilate (200 mg/day) therapy was initiated immediately.Figure 3


Adult-onset Still's disease with disseminated intravascular coagulation and hemophagocytic syndrome: a case report.

Mimura T, Shimodaira M, Kibata M, Tsukadaira A, Shirahata K - BMC Res Notes (2014)

Clinical course of a patient with adult-onset Still’s disease. ALT, alanine transaminase; AST, aspartate transaminase; BT, body temperature; CRP, C-reactive protein; WBC, white blood cell count.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4307634&req=5

Fig3: Clinical course of a patient with adult-onset Still’s disease. ALT, alanine transaminase; AST, aspartate transaminase; BT, body temperature; CRP, C-reactive protein; WBC, white blood cell count.
Mentions: After admission, the high fever persisted, with increasing levels of hepatic and biliary enzymes (Figure 3). On day 4 after admission, the platelet count suddenly decreased to 6.3 × 104/μL, and the fibrinogen level decreased to 122 mg/dL (normal: 190–430 mg/dL). In contrast, the patient had excess fibrin degradation product (FDP; 45.5 μg/mL; normal: 1–10 μg/mL), and a high prothrombin time-international normalized ratio (PT-INR: 1.23; control: 1.0). Based on these data, she was diagnosed with DIC and nafamostat mesilate (200 mg/day) therapy was initiated immediately.Figure 3

Bottom Line: The combination of nafamostat mesilate and prednisolone therapies caused a rapid reduction in the fever and rash.The inflammatory markers decreased immediately, and disseminated intravascular coagulation improved.Her symptoms resolved with low-dose prednisolone treatment, and she was monitored thereafter at our outpatient clinic.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Iida Municipal Hospital, 438 Yawata-machi, Iida, Nagano 395-8502, Japan. masanori19810813@yahoo.co.jp.

ABSTRACT

Background: Adult-onset Still's disease is a rare inflammatory condition of unknown origin characterized by high spiking fever, arthralgia, arthritis, myalgia, salmon-colored evanescent rash, and hepatosplenomegaly. The diagnosis of adult-onset Still's disease requires the exclusion of other possible disorders because it lacks specific clinical and histopathological findings. Adult-onset Still's disease rarely become fatal due to visceral involvements such as disseminated intravascular coagulation.

Case presentation: A 22-year-old Chinese female presented to our medical center with high spiking fever for one week, myalgia for two weeks, and arthralgia and pink maculopapular rash for four weeks. She developed disseminated intravascular coagulation on the fourth day after admission. There was no other explanation for the fever and rash, including infection, malignancy, and collagenosis. Together, the high spiking fever, salmon-colored rash, splenomegaly, and excess hepatic enzyme, indicated adult-onset Still's disease based on the Yamaguchi criteria. Therefore, prednisolone therapy was initiated. The combination of nafamostat mesilate and prednisolone therapies caused a rapid reduction in the fever and rash. The inflammatory markers decreased immediately, and disseminated intravascular coagulation improved. Her symptoms resolved with low-dose prednisolone treatment, and she was monitored thereafter at our outpatient clinic.

Conclusion: The previous use of nonsteroidal anti-inflammatory drugs could have caused disseminated intravascular coagulation in this patient with adult-onset Still's disease. We propose that physicians should consider the possibility of disseminated intravascular coagulation as a complication during the course of adult-onset Still's disease and suggest that prednisolone therapy should be initiated in the early stages of adult-onset Still's disease.

No MeSH data available.


Related in: MedlinePlus