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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.

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Related in: MedlinePlus

Computed tomographic findings of abdomen appear acute acalculous cholecystitis and ileocolitis. (A) A thickened wall of gall bladder with pericholecytic fluid collection, (B) An unenhanced wall thickenings of cecum and terminal ileum.
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Figure 1: Computed tomographic findings of abdomen appear acute acalculous cholecystitis and ileocolitis. (A) A thickened wall of gall bladder with pericholecytic fluid collection, (B) An unenhanced wall thickenings of cecum and terminal ileum.

Mentions: On admission (4th day after the onset), she appeared acutely ill with a confused mental status. Her vital signs were the blood pressure of 90/60 mmHg, the heart rate of 100/min, the temperature of 39.0℃, and the respiratory rate of 30/min. Physical examination revealed facial rash without coalescence, icteric sclera, dehydrated tongue, equivocal neck stiffness, splenomegaly, purpuras over the limbs, scabs of zoster on left forearm and severe tenderness in the right upper and lower quadrant of the abdomen with positive Murphy's sign. There was no definite abdominal rigidity or palpable lymphadenopathy. Initial laboratory results were as follows: WBC 7,100/µL (neutrophil 62%, bands 10%, lymphocyte 15%, monocyte 12%), hemoglobin 9.5g/dL, mean corpuscular volume 74.2 fL (normal 79-95), platelet 17,000/µL, reticulocytes 0.2%, iron 164 µg/dL (normal 50-150), TIBC 218 µg/dL (normal 250-400), ferritin>1,831 ng/mL (normal 10-291), ESR 10 mm/hr, C-reactive protein 16.5 mg/dL (normal 0.1-0.8), total bilirubin 3.7 mg/dL, direct bilirubin 2.8 mg/dL, AST 453 U/L, ALT 154 U/L, ALP 356 U/L, LDH 2,350 U/L, CK 1,547 U/L, BUN 41 mg/dL, creatinine 2.3 mg/dL, total cholesterol 79 mg/dL (normal 130-250), triglyceride 335 mg/dL (normal 50-150), HDL-cholesterol 13 mg/dL (normal 30-70), C3 88.8 mg/dL (normal 79-152) and C4 13.8 mg/dL (normal 16-38). Coagulation tests revealed the following results: PT 18 s (control 12), aPTT 98 s (normal 23-39), fibrinogen 52 mg/dL (normal 190-430), FDP 40 µg/mL (normal<10), antithrombin III 33.9 % (normal 80-120) and D-dimer 4 mg/L (normal<0.3). Direct and indirect Coomb's tests were negative. Blood smear revealed polychromasia, combined with normocytic and microcytic anemia and severe thrombocytopenia. Antibodies to nuclear antigens, dsDNA, cardiolipin, VDRL and rheumatoid factor were negative. Urine examination revealed protein 1.41 g/day with granular casts, fractional excretion of sodium measuring 1% and urine sodium concentration 21 mmol/L. Antistreptolysin O and Widal test were normal. Hepatitis B surface antigen, hepatitis C virus, and HIV antibodies were negative. Serological tests for varicella-zoster virus (VZV), Epstein-Barr virus, cytomegalovirus and herpes simplex viruses revealed no signs of recent infections. Chest radiograph and electrocardiogram appeared normal. Abdominal computed tomography (CT) images showed acute acalculous cholecystitis, ileocolitis (Fig. 1), hepatosplenomegaly, a small amount of right pleural effusion and ascites. The patient had no history of recurrent episode of abdominal pain or diarrhea. Bone marrow (BM) biopsy and cerebrospinal fluid examination were not performed due to hemorrhagic diathesis.


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)

Computed tomographic findings of abdomen appear acute acalculous cholecystitis and ileocolitis. (A) A thickened wall of gall bladder with pericholecytic fluid collection, (B) An unenhanced wall thickenings of cecum and terminal ileum.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Computed tomographic findings of abdomen appear acute acalculous cholecystitis and ileocolitis. (A) A thickened wall of gall bladder with pericholecytic fluid collection, (B) An unenhanced wall thickenings of cecum and terminal ileum.
Mentions: On admission (4th day after the onset), she appeared acutely ill with a confused mental status. Her vital signs were the blood pressure of 90/60 mmHg, the heart rate of 100/min, the temperature of 39.0℃, and the respiratory rate of 30/min. Physical examination revealed facial rash without coalescence, icteric sclera, dehydrated tongue, equivocal neck stiffness, splenomegaly, purpuras over the limbs, scabs of zoster on left forearm and severe tenderness in the right upper and lower quadrant of the abdomen with positive Murphy's sign. There was no definite abdominal rigidity or palpable lymphadenopathy. Initial laboratory results were as follows: WBC 7,100/µL (neutrophil 62%, bands 10%, lymphocyte 15%, monocyte 12%), hemoglobin 9.5g/dL, mean corpuscular volume 74.2 fL (normal 79-95), platelet 17,000/µL, reticulocytes 0.2%, iron 164 µg/dL (normal 50-150), TIBC 218 µg/dL (normal 250-400), ferritin>1,831 ng/mL (normal 10-291), ESR 10 mm/hr, C-reactive protein 16.5 mg/dL (normal 0.1-0.8), total bilirubin 3.7 mg/dL, direct bilirubin 2.8 mg/dL, AST 453 U/L, ALT 154 U/L, ALP 356 U/L, LDH 2,350 U/L, CK 1,547 U/L, BUN 41 mg/dL, creatinine 2.3 mg/dL, total cholesterol 79 mg/dL (normal 130-250), triglyceride 335 mg/dL (normal 50-150), HDL-cholesterol 13 mg/dL (normal 30-70), C3 88.8 mg/dL (normal 79-152) and C4 13.8 mg/dL (normal 16-38). Coagulation tests revealed the following results: PT 18 s (control 12), aPTT 98 s (normal 23-39), fibrinogen 52 mg/dL (normal 190-430), FDP 40 µg/mL (normal<10), antithrombin III 33.9 % (normal 80-120) and D-dimer 4 mg/L (normal<0.3). Direct and indirect Coomb's tests were negative. Blood smear revealed polychromasia, combined with normocytic and microcytic anemia and severe thrombocytopenia. Antibodies to nuclear antigens, dsDNA, cardiolipin, VDRL and rheumatoid factor were negative. Urine examination revealed protein 1.41 g/day with granular casts, fractional excretion of sodium measuring 1% and urine sodium concentration 21 mmol/L. Antistreptolysin O and Widal test were normal. Hepatitis B surface antigen, hepatitis C virus, and HIV antibodies were negative. Serological tests for varicella-zoster virus (VZV), Epstein-Barr virus, cytomegalovirus and herpes simplex viruses revealed no signs of recent infections. Chest radiograph and electrocardiogram appeared normal. Abdominal computed tomography (CT) images showed acute acalculous cholecystitis, ileocolitis (Fig. 1), hepatosplenomegaly, a small amount of right pleural effusion and ascites. The patient had no history of recurrent episode of abdominal pain or diarrhea. Bone marrow (BM) biopsy and cerebrospinal fluid examination were not performed due to hemorrhagic diathesis.

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