Limits...
Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A.

Park JH, Bae JH, Choi YS, Lee HS, Jun JB, Jung S, Yoo DH, Bae SC, Kim TH - J. Korean Med. Sci. (2004)

Bottom Line: The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain.After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly.Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.

View Article: PubMed Central - PubMed

Affiliation: Division of Rheumatology, The Hospital for Rheumatic Diseases, Hanyang University Medical Center, Seoul, Korea.

ABSTRACT
Severe systemic manifestations of adult onset Still's disease (AOSD) are often fatal and occasionally related to hemophagocytic syndrome (HS). We describe the case of a 49-yr-old woman with AOSD presenting with non-remitting high fever, confusion, jaundice, hepatosplenomegaly, serositis, azotemia, pancytopenia, coagulopathy with disseminated intravascular coagulation (DIC), hyperferritinemia, acute acalculous cholecystitis and ileocolitis noted in computed tomographic images. The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain. Although bone marrow examination was not performed due to hemorrhagic diathesis, we suspected AOSD-associated HS on the basis of clinical course without detectable infectious agents in cultures or serologic studies. Intravenous immunoglobulin, pulse methylprednisolone, oral cyclosporine A (CsA) and ceftriaxone brought about transient improvement of fever and confusion, but the disease progressed. After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly. Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.

Show MeSH

Related in: MedlinePlus

Clinical course and treatment of this patient. FFP, fresh frozen plasma; PC, platelet concentrate; PRC, packed red cell; PSL, prednisolone; IVIG, intravenous immunoglobulin; ND, not done.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC2822251&req=5

Figure 2: Clinical course and treatment of this patient. FFP, fresh frozen plasma; PC, platelet concentrate; PRC, packed red cell; PSL, prednisolone; IVIG, intravenous immunoglobulin; ND, not done.

Mentions: The combination of anemia, thrombocytopenia, coagulopathy, DIC, hepatic and renal dysfunctions, neurological symptoms, and capillary leakage signs was compatible with severe systemic inflammatory response syndrome. A differential diagnosis of hemophagocytic syndrome and septic DIC was considered. Fluid and electrolytes replacement, fresh frozen plasma and platelet concentrates transfusions were immediately initiated. Empirical antibiotics (ceftriaxone, 3 g/day) and intravenous immunoglobulin (IVIG, 400 mg/kg/day) therapy were also initiated. On the next day after the admission, confused mentality diminished, and serum creatinine level was normalized. Negative results for blood, throat, urine and stool cultures were noted. On the basis of patient's clinical presentations, marked elevated ferritin, hypertriglyceridemia, and no evidence of infectious etiology, a presumptive diagnosis of reactive hemophagocytic syndrome (RHS) associated with AOSD was made. Intravenous pulsed methylprednisolone (Methyl-PSL, 1 g/day) and oral cyclosporine A (CsA, 2 mg/kg/day) were added. Two day after the admission, fever was disappeared rapidly, and abdominal tenderness was also decreased. On fourth day of the admission, high fever and neurological dysfunctions such as confusion, irritability, disorientation abruptly recurred, and abdominal tenderness re-aggravated despite treatment with IVIG (for 3 days), pulsed methyl-PSL (for 3 days) followed by methyl-PSL 62.5 mg/day, continuous oral CsA and blood component replacements. Although levels of transaminase and LDH were gradually decreased, pancytopenia and DIC progressed. On the sixth day of the admission, we increased the dosage of oral CsA from 2 mg/kg/day to 3 mg/kg/day. Fever and confusion were disappeared dramatically within a day. She no longer needed any transfusion, with pancytopenia and DIC rapidly improving. Subsequently, abdominal tenderness also disappeared within 3 days. On day 42 of the hospitalization, all laboratory abnormalities except lipid profile returned to normal (Fig. 2). Previous radiological abnormalities were also normalized on follow-up study. CsA administration was stopped, and PSL was tapered. She has remained clinically well with low-dose PSL alone for over one year.


Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A.

Park JH, Bae JH, Choi YS, Lee HS, Jun JB, Jung S, Yoo DH, Bae SC, Kim TH - J. Korean Med. Sci. (2004)

Clinical course and treatment of this patient. FFP, fresh frozen plasma; PC, platelet concentrate; PRC, packed red cell; PSL, prednisolone; IVIG, intravenous immunoglobulin; ND, not done.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2822251&req=5

Figure 2: Clinical course and treatment of this patient. FFP, fresh frozen plasma; PC, platelet concentrate; PRC, packed red cell; PSL, prednisolone; IVIG, intravenous immunoglobulin; ND, not done.
Mentions: The combination of anemia, thrombocytopenia, coagulopathy, DIC, hepatic and renal dysfunctions, neurological symptoms, and capillary leakage signs was compatible with severe systemic inflammatory response syndrome. A differential diagnosis of hemophagocytic syndrome and septic DIC was considered. Fluid and electrolytes replacement, fresh frozen plasma and platelet concentrates transfusions were immediately initiated. Empirical antibiotics (ceftriaxone, 3 g/day) and intravenous immunoglobulin (IVIG, 400 mg/kg/day) therapy were also initiated. On the next day after the admission, confused mentality diminished, and serum creatinine level was normalized. Negative results for blood, throat, urine and stool cultures were noted. On the basis of patient's clinical presentations, marked elevated ferritin, hypertriglyceridemia, and no evidence of infectious etiology, a presumptive diagnosis of reactive hemophagocytic syndrome (RHS) associated with AOSD was made. Intravenous pulsed methylprednisolone (Methyl-PSL, 1 g/day) and oral cyclosporine A (CsA, 2 mg/kg/day) were added. Two day after the admission, fever was disappeared rapidly, and abdominal tenderness was also decreased. On fourth day of the admission, high fever and neurological dysfunctions such as confusion, irritability, disorientation abruptly recurred, and abdominal tenderness re-aggravated despite treatment with IVIG (for 3 days), pulsed methyl-PSL (for 3 days) followed by methyl-PSL 62.5 mg/day, continuous oral CsA and blood component replacements. Although levels of transaminase and LDH were gradually decreased, pancytopenia and DIC progressed. On the sixth day of the admission, we increased the dosage of oral CsA from 2 mg/kg/day to 3 mg/kg/day. Fever and confusion were disappeared dramatically within a day. She no longer needed any transfusion, with pancytopenia and DIC rapidly improving. Subsequently, abdominal tenderness also disappeared within 3 days. On day 42 of the hospitalization, all laboratory abnormalities except lipid profile returned to normal (Fig. 2). Previous radiological abnormalities were also normalized on follow-up study. CsA administration was stopped, and PSL was tapered. She has remained clinically well with low-dose PSL alone for over one year.

Bottom Line: The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain.After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly.Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.

View Article: PubMed Central - PubMed

Affiliation: Division of Rheumatology, The Hospital for Rheumatic Diseases, Hanyang University Medical Center, Seoul, Korea.

ABSTRACT
Severe systemic manifestations of adult onset Still's disease (AOSD) are often fatal and occasionally related to hemophagocytic syndrome (HS). We describe the case of a 49-yr-old woman with AOSD presenting with non-remitting high fever, confusion, jaundice, hepatosplenomegaly, serositis, azotemia, pancytopenia, coagulopathy with disseminated intravascular coagulation (DIC), hyperferritinemia, acute acalculous cholecystitis and ileocolitis noted in computed tomographic images. The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain. Although bone marrow examination was not performed due to hemorrhagic diathesis, we suspected AOSD-associated HS on the basis of clinical course without detectable infectious agents in cultures or serologic studies. Intravenous immunoglobulin, pulse methylprednisolone, oral cyclosporine A (CsA) and ceftriaxone brought about transient improvement of fever and confusion, but the disease progressed. After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly. Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.

Show MeSH
Related in: MedlinePlus