å¡ CASE REPORT å¡ Disseminated Intravascular Coagulation in Lupus Erythematosus with Acute Liver Damage Yoshinori Shimamoto, Masayuki Sano, Shoko Kaneda, Seiji Tsunada, Kyosuke Yamamoto and Masaya Yamaguchi A case of disseminated intravascular coagulation (DIC) in a patient with systemic lupus erythematosus (SLE) with acute liver dysfunction is described. A 37-year-old man with SLE developed acute DIC and marked liver damage after fracture of the right clavicle and pharyngitis. Treatment with high-dose steroids, heparin, antithrombin III, gabexate mesilate, and antibiotics resulted in prompt improvement. The recovery of an SLE patient after acute DIC and marked liver damage is considered very rare. We report here such a case and discuss (Internal Medicine 31: 1392-1395, 1992) the previous reports. Key words: autoimmune disease, consumption coagulopathy, liver function
Introduction Although systemic lupus erythematosus (SLE) was not cited as an associated or causative illness in two large studies of acute disseminated intravascular coagulation (DIC) (1, 2), the occurrence of DIC in SLE patients was initially reported by McKay (3), and five subsequent reports have followed (4-8). In these cases, however, the recently developed assays for coagulation tests such as thrombin-antithrombin III complex (TAT), D-dimer, and plasmin-O2 plasmin-inhibitor complex (PIC) were not
On May 13, 1990, he suffered a fractured right clavicle when he was playing baseball and underwent an operation on May 22. Thereafter, he was given antibiotics (cefazoline sodium at lg/day, followed by cefuroxime axetil at 750mg/day). On June 3 he developed a high fever and sore throat, and was transferred to our hospital. At that time, gingival and nasal bleeding were found. On exam ination, the patient presented facial rash, reddish throat, and hepatomegaly. The chest X-ray revealed right pleural effusion. The hematological studies revealed that WBC was 4,500/mm3, hemoglobin 15.6 g/dl, and platelet
count 8,000/mm3. The coagulation tests indicated pro longed prothrombin time and activated partial thrombin time. Antithrombin III measured by the method em ploying the thrombin-specific chromatogenic substrate (9) and fibrinogen were decreased. TAT, D-dimer, and PIC determined by enzyme-linked immunosorbent assays (10-12) and FDP-E (fibrin/fibrinogen degradation No lupus anticoagulant The product-E) measured by(14) the was latexdemonstrated. photometric immuno immunological studies revealed low serum complement assay (13) were markedly increased, as shown in Table 1. levels, and high levels of anti-double-stranded DNA (15) and antinuclear antibody. Extremely high levels of trans paration, increased antinuclear antibody, increased aminase and LDH were observed in the absence of DNA binding antibody, and low serum complement hyperbilirubinemia (Table 2). The serum level of crea levels. He was treated with prednisone and the symptoms disappeared. After prednisone was discontinued in 1982, tinine phosphokinase (CPK) was normal. There was no he had no symptoms and was followed as an outpatient. serologic evidence of hepatitis A, B, or C viruses. He From the Department of Internal Medicine, Saga Medical School, Saga Received for publication May 25, 1992; Accepted for publication October 21, 1992 Reprint requests should be addressed to Dr. Yoshinori Shimamoto, the Department of Internal Medicine, Saga Medical School, Nabeshi 849,Japan performed. Moreover, the recovery of an SLE patient after acute DIC and marked liver damage has not been reported yet. We report such a case and discuss the preceding Case Reportevents, lupus activity, complication, treatment and outcome, as well as those in previous reports. In 1981, a 37-year-old man was first seen at our hospital with the complaints of purpura and gingival bleeding. SLE was diagnosed on the basis of severe thrombocytopenia (7,000/mm3), positive LE cell pre
1392
Internal
Medicine
Vol. 31, No. 12 (December
1992)
DIC in SLE Table 1. Coagulation Profiles of the Patient before, during, and after Development of Acute DIC T est
H em o glob in P latelet PT A PTT F ibrino gen A ntithrom bin III TA T F D P -E D -dim er P IC
N o rm al R ange
(M Barch efo re28)
(Ju D ur ne i n 7) g
(Jun A ft eer 25)
12 - 17 g/d1 13 - 35 x lO4/m m 3 /con tro lsec /con trol sec 170- 4 10 m g/d1 79- 12 1% < 3 .O n g/m l < 100 n g/m l < 150 ng /m l < 0 .8 ^g/m l
14 .7 15 .2 14 .2/15 .4 47 .2/44.5 194 NT NT NT NT NT
15.6 0.8 20.0 /15 .6 135.5/44 .5 95 55 330 14 ,225 5 ,720 ll.2
10 .5 13 .4 13 .2/15 .2 39 .8/44 .3 176 116 2 .9 309 127 1.2
PT: prothrombin time, APTT: activated partial thromboplastin time , TAT: thrombin-antithromin III complex, PIC: plasmin-a2 plasmin-inhibitor complex, NT: not tested. Table 2. Serological and Liver Function Tests of the Patient before, during, and after Development of Acute DIC T est C H 50 C3 C4 L E cell A n ti-D N A A b ANA W BC H em atu ri a P ro teinuria T .P . A lb um in T .B . G OT G PT LDH A LP LA P y-G T P CRP
N o rm al R an ge
B efore (M arch 28 )
30- 40 C H 50 /m 1 60- 130 m g/d1 14 - 50 m g/d1
30 52 20
16 22 12
33 65 16
14 .8 10 6 .12
10 .5 40 4 .5
7 .5 40 9 .3
8 .O g/d1 5 .2 g/d1 1 .O m g /d1 35 IU /1 40/IU /1 520 IU /1 482 IU /1 78 IU /1
8 .2 4 .6 0 .5 19 13 251 164 53
6 .7 3 .4 1.0 4 ,530 2 ,292 10 ,125 533 262
6 .6 3 .7 0 .8 33 82 29 6 195 127
0 - 50 IU /1 0 - 0.5 m g/d 1
12 0 .2
172 9 .7
12 1 0.1
< 7 IU /m 1 < 10 4.5 - 7.O x lO3/m m 3
6 .53 .10 .2 10 0120 122 37 -
D urin g (Ju ne 7)
A fter (June 25)
ANA: antinuclear antibody, WBC: white blood cell count, T.P.: total protein, T.B.: total bilirubin, GOT: glutamic oxaloacetate transaminase, GPT: glutamic pyruvate transaminase, LDH: lactate dehydrogenase, ALP: alkaline phosphatase, y-GTP: y-glutamyl transpeptidase. was diagnosed according to the lupus activity criteria count (16) as having active SLE complicated with acute DIC and marked liver damage. He was immediately treated with high-dose steroid therapy consisting of meth ylprednisolone at 1 g/day for SLE, heparin (10,000 U/day) simultaneously with antithrombin III (1 ,500 concentrate units/day) and gabexate mesilate (2,000mg/day) for acute DIC, and antibiotics for possible infection, although several bacteriological cultures from blood, throat, urine and sputum were negative for pathogens. He recovered soon after the treatment. The laboratory data before, during, and after the development of acute DIC are Internal shown Medicine in TablesVol.1 and 31, 2. No.Liver 12 (December biopsy carried 1992) out on July
19, 1990 revealed slight fibrosis and lymphocyte infil tration in the portal area, and deposits of hemosiderin. Thereafter, low-dose steroid therapy was maintained, and, as of July 1992, the patient was well with normal liver function tests. Discussion The reported clinical features of SLE patients who developed acute DIC (3-8), including the present patient, are summarized in Table 3. The development of DIC in SLE patients is rare. The preceding events in our patient included bone fracture, operation, and 1393
Shimamoto et al Table 3. C ase
A ge/S ex
D uratio n of S L E
P rece din g even ts
38/F
14 yr
26/M
4 yr
cho lecystitis cho lecyste ctom y ph aryn gitis skin b iop sy pharyn gitis
19/F
ND
40/F
10
3 yr
48/M
ND
57/F
ND
Reported
Cases of SLE with Acute DIC
C om plicatio n
L up us activity
n ep hritis
ND
n ep hritis
activ e
ND
active
ND
in terstitial pn eum onitis
ND
ND
pu lm o nary fi b ro sis ND
ND active
skin bio psy
23/F
ly r
ND
pancreatitis
active
25/F
6yr
ND
ND
active
33 /F
14 y r
ND
neph ritis,
ND
37 /M
9yr
acute liver d am age
active
ND: not described,
yr: year(s),
fracture , op eration , ph aryngitis
T reatm en t
O utco m e
antibiotics
died
antibiotics, steroid antibiotics, steroid , hep arin antibio tics, stero id ,im m un osupp ressan t, F F P stero id , FFP an tibio tics, stero id , h eparin an tibio tics, steroid , h eparin an tibio tics, steroid , h eparin an tib io tics, steroid , h eparin , an tib io tics, steroid ,h eparin , gab exate
im p roved
R eference
im p roved d ied im pro ved im pro ved im pro ved im prov ed im p rove d im prove d
presen t
FFP: fresh frozen plasma.
pharyngitis. Surgical procedures are reported to be pre ceding events in SLE patients with DIC; cholecystectomy for chronic cholecystitis (3) and skin biopsy (4, 8) have induced DIC. As in our case, pharyngitis has also pre ceded DIC in the previously reported patients (4, 5). The reported complications of SLE patients with DIC included nephritis (3, 4, 8), interstitial pneumonitis (6), pulmonary fibrosis (7), and pancreatitis (8). The occur rence of marked liver damage in these patients has not been reported. One explanation for the acute liver damage in our patient may be the relationship between active SLE vasculitis and the development of liver damage. However, this explanation is unlikely, because systemic vasculitis in SLE apparently does not affect only one organ. The possibility that our patient had acute exacerbation of chronic active hepatitis (17) is also unlikely, because the liver damage in our patient was transient and because the liver biopsy revealed no evi dence of chronic active hepatitis. Significant liver disease is reported to be rare in SLE (18, 19). Although some cases of liver disease in SLE patients have been reported, these patients, unlike our patient, showed chronic course and moderate or mild liver dysfunction (20). It is poss ible that in our patient a virus other than hepatitis A, B, or C caused the pharyngitis and induced marked liver damage and acute DIC. Another possibility is that the antibiotics used after the operation in the present patient caused drug-induced liver damage, and that the acute DIC was independently induced by the bone fracture 1394 and operation.
In most of the patients, including our patient, the SLE was active according to the lupus activity criteria count (16) and was improved by the treatment. Combination therapy with heparin for DIC, high-dose steroid therapy for SLE, and antibiotics for possible infection was the most common treatment in these patients. Although there was no evidence of infection, most were given antibiotics. We recommend that antimicrobial agents be administered to these patients until infection has been Recently-developed assays for coagulation study, such ruled out. as TAT, D-dimer, and PIC, were conducted in our case; such data have not appeared in previous reports of SLE patients with DIC. The clotting system of the blood is known to be abnormal in SLE patients (14), and moder ate elevation of the PIC level has been demonstrated in those with active SLE vasculitis (12). Our patient showed an extremely high level of PIC, which is thought to have been the result of acute DIC. DIC in SLE is unusual, and coagulation studies including newly available assays are necessary to elucidate the pathophysiology as such cases accumulate. References Siegal T, Seligsohn U, Aghai E, Modan M. Clinical and laboratory aspects of disseminated intravascular coagulation (DIC) : A study of 118 cases. Thromb Haemost 39: 122, 1978. Bick RL. Disseminated intravascular coagulation and related syndrome , etiology, pathophysiology, diagnosis and management. Am J Hematol 5: 265, 1978. Internal
Medicine
Vol. 31, No. 12 (December
1992)
DIC in SLE 3) 4)
McKay DG. Disease of hypersensitivity: Disseminated intravascular coagulation. Arch Int Med 116: 83, 1965. Whitaker AN, EmmersonBT. Case report: Acute disseminated intravascular coagulation in systemic lupus erythematosus. Aust NZJMed
5) 6) 7)
8)
9)
10)
ll)
12)
2: 182,
1972.
Beal CL, Pierce LE. Case report: Intravascular coagulation in acute lupus erythematosus. JAMA 234: 518, 1975. Chellingsworth M, Scott DGI. Acute systemic lupus erythematosus with fatal pneumonitis and disseminated intravascular coagulation. Ann Rheum Dis 44: 67, 1985. Riddell SR, Shojania M. Disseminated intravascular coagulation in lupus erythematosus responding to prednisone therapy. Am J Hematol 23: 65, 1985. Kerr LD, Spiera H, Aledort LN. Acute disseminated intravascular coagulation as a complication of systemic lupus erythematosus. NY State J Med 87: 181, 1987. Scully NF, Kakkar W. Methods for semi micro or automated determination of thrombin, antithrombin, and heparin co factor using the substrate, H-D-Phe-Pip-Arg-p-NITROANILIDE-2HCl. Clin Chim Acta 79: 595, 1977. Pelzer H, Schwarz A, Heimburger N. Determination of human thrombin-antithrombin III complex in plasma with an enzymelinked immunosorbent assay. Thromb Haemost 59: 101, 1988. Elms MJ, Bunce IH, Bundesen PG, et al. Measurement of crosslinked fibrin degradation products - animmunoassay using monoclonal antibodies. Thromb Haemost 50: 591, 1983. Kawakami M, Kawagoe M, Harigai M, et al. Elevated plasma
Internal
Medicine
Vol. 31, No. 12 (December
1992)
levels of a^-plasmin inhibitor-plasmin complex in patients with rheumatic disease. Possible role of fibrinolytic mechanism in vasculitis. Arthritis Rheum 32: 1427, 1989. Sudo T, Sawai M. Immunoreactivities of fibrin (ogen) related antigens and antibodies in radioimmunoassay and latex photo metric immunoassay. Acta Haematol Jpn 44: 131, 1981. Feinstein DI, Rapaport SI. Acquired inhibitors of blood coagu lation. Prog Hemost Thromb 1: 75, 1972. Feltkamp T, Kirkwood T, Maini R, Aarden L. The first inter national standard for antibodies to double stranded DNA. Ann Rheum Dis 47: 740, 1988. Urowitz MB, Gladman DD, Tozman ECS, Goldsmith CH. The lupus activity criteria count (LACC). J Rheumatol ll: 783, 1984. Gurian LE, Rogoff TM, Ware AJ, et al. The immunologic diagnosis of chronic active "autoimmune" hepatitis: Distinction from systemic lupus erythematosus. Hepatology 5: 397, 1985. Decker JL, Steinberg AD, Gershwin ME, et al. Systemic lupus erythematosus - Contrasts and comparisons. Ann Intern Med 82: 391, 1975. Rothfield NF. Systemic lupus erythematosus. in: Rheumatic Diseases: Diagnosis and Management, Katz WA, Ed. Philadelphia, JB Lipincott Co, p765, 1977. Runyon BA, LaBrecque DR, Anuras S. The spectrum of liver disease in systemic lupus erythematosus. Report of 33 histologically proved cases and review of the literature. Am J Med 69: 187, 1980.
1395
Introduction Although systemic lupus erythematosus (SLE) was not cited as an associated or causative illness in two large studies of acute disseminated intravascular coagulation (DIC) (1, 2), the occurrence of DIC in SLE patients was initially reported by McKay (3), and five subsequent reports have followed (4-8). In these cases, however, the recently developed assays for coagulation tests such as thrombin-antithrombin III complex (TAT), D-dimer, and plasmin-O2 plasmin-inhibitor complex (PIC) were not
On May 13, 1990, he suffered a fractured right clavicle when he was playing baseball and underwent an operation on May 22. Thereafter, he was given antibiotics (cefazoline sodium at lg/day, followed by cefuroxime axetil at 750mg/day). On June 3 he developed a high fever and sore throat, and was transferred to our hospital. At that time, gingival and nasal bleeding were found. On exam ination, the patient presented facial rash, reddish throat, and hepatomegaly. The chest X-ray revealed right pleural effusion. The hematological studies revealed that WBC was 4,500/mm3, hemoglobin 15.6 g/dl, and platelet
count 8,000/mm3. The coagulation tests indicated pro longed prothrombin time and activated partial thrombin time. Antithrombin III measured by the method em ploying the thrombin-specific chromatogenic substrate (9) and fibrinogen were decreased. TAT, D-dimer, and PIC determined by enzyme-linked immunosorbent assays (10-12) and FDP-E (fibrin/fibrinogen degradation No lupus anticoagulant The product-E) measured by(14) the was latexdemonstrated. photometric immuno immunological studies revealed low serum complement assay (13) were markedly increased, as shown in Table 1. levels, and high levels of anti-double-stranded DNA (15) and antinuclear antibody. Extremely high levels of trans paration, increased antinuclear antibody, increased aminase and LDH were observed in the absence of DNA binding antibody, and low serum complement hyperbilirubinemia (Table 2). The serum level of crea levels. He was treated with prednisone and the symptoms disappeared. After prednisone was discontinued in 1982, tinine phosphokinase (CPK) was normal. There was no he had no symptoms and was followed as an outpatient. serologic evidence of hepatitis A, B, or C viruses. He From the Department of Internal Medicine, Saga Medical School, Saga Received for publication May 25, 1992; Accepted for publication October 21, 1992 Reprint requests should be addressed to Dr. Yoshinori Shimamoto, the Department of Internal Medicine, Saga Medical School, Nabeshi 849,Japan performed. Moreover, the recovery of an SLE patient after acute DIC and marked liver damage has not been reported yet. We report such a case and discuss the preceding Case Reportevents, lupus activity, complication, treatment and outcome, as well as those in previous reports. In 1981, a 37-year-old man was first seen at our hospital with the complaints of purpura and gingival bleeding. SLE was diagnosed on the basis of severe thrombocytopenia (7,000/mm3), positive LE cell pre
1392
Internal
Medicine
Vol. 31, No. 12 (December
1992)
DIC in SLE Table 1. Coagulation Profiles of the Patient before, during, and after Development of Acute DIC T est
H em o glob in P latelet PT A PTT F ibrino gen A ntithrom bin III TA T F D P -E D -dim er P IC
N o rm al R ange
(M Barch efo re28)
(Ju D ur ne i n 7) g
(Jun A ft eer 25)
12 - 17 g/d1 13 - 35 x lO4/m m 3 /con tro lsec /con trol sec 170- 4 10 m g/d1 79- 12 1% < 3 .O n g/m l < 100 n g/m l < 150 ng /m l < 0 .8 ^g/m l
14 .7 15 .2 14 .2/15 .4 47 .2/44.5 194 NT NT NT NT NT
15.6 0.8 20.0 /15 .6 135.5/44 .5 95 55 330 14 ,225 5 ,720 ll.2
10 .5 13 .4 13 .2/15 .2 39 .8/44 .3 176 116 2 .9 309 127 1.2
PT: prothrombin time, APTT: activated partial thromboplastin time , TAT: thrombin-antithromin III complex, PIC: plasmin-a2 plasmin-inhibitor complex, NT: not tested. Table 2. Serological and Liver Function Tests of the Patient before, during, and after Development of Acute DIC T est C H 50 C3 C4 L E cell A n ti-D N A A b ANA W BC H em atu ri a P ro teinuria T .P . A lb um in T .B . G OT G PT LDH A LP LA P y-G T P CRP
N o rm al R an ge
B efore (M arch 28 )
30- 40 C H 50 /m 1 60- 130 m g/d1 14 - 50 m g/d1
30 52 20
16 22 12
33 65 16
14 .8 10 6 .12
10 .5 40 4 .5
7 .5 40 9 .3
8 .O g/d1 5 .2 g/d1 1 .O m g /d1 35 IU /1 40/IU /1 520 IU /1 482 IU /1 78 IU /1
8 .2 4 .6 0 .5 19 13 251 164 53
6 .7 3 .4 1.0 4 ,530 2 ,292 10 ,125 533 262
6 .6 3 .7 0 .8 33 82 29 6 195 127
0 - 50 IU /1 0 - 0.5 m g/d 1
12 0 .2
172 9 .7
12 1 0.1
< 7 IU /m 1 < 10 4.5 - 7.O x lO3/m m 3
6 .53 .10 .2 10 0120 122 37 -
D urin g (Ju ne 7)
A fter (June 25)
ANA: antinuclear antibody, WBC: white blood cell count, T.P.: total protein, T.B.: total bilirubin, GOT: glutamic oxaloacetate transaminase, GPT: glutamic pyruvate transaminase, LDH: lactate dehydrogenase, ALP: alkaline phosphatase, y-GTP: y-glutamyl transpeptidase. was diagnosed according to the lupus activity criteria count (16) as having active SLE complicated with acute DIC and marked liver damage. He was immediately treated with high-dose steroid therapy consisting of meth ylprednisolone at 1 g/day for SLE, heparin (10,000 U/day) simultaneously with antithrombin III (1 ,500 concentrate units/day) and gabexate mesilate (2,000mg/day) for acute DIC, and antibiotics for possible infection, although several bacteriological cultures from blood, throat, urine and sputum were negative for pathogens. He recovered soon after the treatment. The laboratory data before, during, and after the development of acute DIC are Internal shown Medicine in TablesVol.1 and 31, 2. No.Liver 12 (December biopsy carried 1992) out on July
19, 1990 revealed slight fibrosis and lymphocyte infil tration in the portal area, and deposits of hemosiderin. Thereafter, low-dose steroid therapy was maintained, and, as of July 1992, the patient was well with normal liver function tests. Discussion The reported clinical features of SLE patients who developed acute DIC (3-8), including the present patient, are summarized in Table 3. The development of DIC in SLE patients is rare. The preceding events in our patient included bone fracture, operation, and 1393
Shimamoto et al Table 3. C ase
A ge/S ex
D uratio n of S L E
P rece din g even ts
38/F
14 yr
26/M
4 yr
cho lecystitis cho lecyste ctom y ph aryn gitis skin b iop sy pharyn gitis
19/F
ND
40/F
10
3 yr
48/M
ND
57/F
ND
Reported
Cases of SLE with Acute DIC
C om plicatio n
L up us activity
n ep hritis
ND
n ep hritis
activ e
ND
active
ND
in terstitial pn eum onitis
ND
ND
pu lm o nary fi b ro sis ND
ND active
skin bio psy
23/F
ly r
ND
pancreatitis
active
25/F
6yr
ND
ND
active
33 /F
14 y r
ND
neph ritis,
ND
37 /M
9yr
acute liver d am age
active
ND: not described,
yr: year(s),
fracture , op eration , ph aryngitis
T reatm en t
O utco m e
antibiotics
died
antibiotics, steroid antibiotics, steroid , hep arin antibio tics, stero id ,im m un osupp ressan t, F F P stero id , FFP an tibio tics, stero id , h eparin an tibio tics, steroid , h eparin an tibio tics, steroid , h eparin an tib io tics, steroid , h eparin , an tib io tics, steroid ,h eparin , gab exate
im p roved
R eference
im p roved d ied im pro ved im pro ved im pro ved im prov ed im p rove d im prove d
presen t
FFP: fresh frozen plasma.
pharyngitis. Surgical procedures are reported to be pre ceding events in SLE patients with DIC; cholecystectomy for chronic cholecystitis (3) and skin biopsy (4, 8) have induced DIC. As in our case, pharyngitis has also pre ceded DIC in the previously reported patients (4, 5). The reported complications of SLE patients with DIC included nephritis (3, 4, 8), interstitial pneumonitis (6), pulmonary fibrosis (7), and pancreatitis (8). The occur rence of marked liver damage in these patients has not been reported. One explanation for the acute liver damage in our patient may be the relationship between active SLE vasculitis and the development of liver damage. However, this explanation is unlikely, because systemic vasculitis in SLE apparently does not affect only one organ. The possibility that our patient had acute exacerbation of chronic active hepatitis (17) is also unlikely, because the liver damage in our patient was transient and because the liver biopsy revealed no evi dence of chronic active hepatitis. Significant liver disease is reported to be rare in SLE (18, 19). Although some cases of liver disease in SLE patients have been reported, these patients, unlike our patient, showed chronic course and moderate or mild liver dysfunction (20). It is poss ible that in our patient a virus other than hepatitis A, B, or C caused the pharyngitis and induced marked liver damage and acute DIC. Another possibility is that the antibiotics used after the operation in the present patient caused drug-induced liver damage, and that the acute DIC was independently induced by the bone fracture 1394 and operation.
In most of the patients, including our patient, the SLE was active according to the lupus activity criteria count (16) and was improved by the treatment. Combination therapy with heparin for DIC, high-dose steroid therapy for SLE, and antibiotics for possible infection was the most common treatment in these patients. Although there was no evidence of infection, most were given antibiotics. We recommend that antimicrobial agents be administered to these patients until infection has been Recently-developed assays for coagulation study, such ruled out. as TAT, D-dimer, and PIC, were conducted in our case; such data have not appeared in previous reports of SLE patients with DIC. The clotting system of the blood is known to be abnormal in SLE patients (14), and moder ate elevation of the PIC level has been demonstrated in those with active SLE vasculitis (12). Our patient showed an extremely high level of PIC, which is thought to have been the result of acute DIC. DIC in SLE is unusual, and coagulation studies including newly available assays are necessary to elucidate the pathophysiology as such cases accumulate. References Siegal T, Seligsohn U, Aghai E, Modan M. Clinical and laboratory aspects of disseminated intravascular coagulation (DIC) : A study of 118 cases. Thromb Haemost 39: 122, 1978. Bick RL. Disseminated intravascular coagulation and related syndrome , etiology, pathophysiology, diagnosis and management. Am J Hematol 5: 265, 1978. Internal
Medicine
Vol. 31, No. 12 (December
1992)
DIC in SLE 3) 4)
McKay DG. Disease of hypersensitivity: Disseminated intravascular coagulation. Arch Int Med 116: 83, 1965. Whitaker AN, EmmersonBT. Case report: Acute disseminated intravascular coagulation in systemic lupus erythematosus. Aust NZJMed
5) 6) 7)
8)
9)
10)
ll)
12)
2: 182,
1972.
Beal CL, Pierce LE. Case report: Intravascular coagulation in acute lupus erythematosus. JAMA 234: 518, 1975. Chellingsworth M, Scott DGI. Acute systemic lupus erythematosus with fatal pneumonitis and disseminated intravascular coagulation. Ann Rheum Dis 44: 67, 1985. Riddell SR, Shojania M. Disseminated intravascular coagulation in lupus erythematosus responding to prednisone therapy. Am J Hematol 23: 65, 1985. Kerr LD, Spiera H, Aledort LN. Acute disseminated intravascular coagulation as a complication of systemic lupus erythematosus. NY State J Med 87: 181, 1987. Scully NF, Kakkar W. Methods for semi micro or automated determination of thrombin, antithrombin, and heparin co factor using the substrate, H-D-Phe-Pip-Arg-p-NITROANILIDE-2HCl. Clin Chim Acta 79: 595, 1977. Pelzer H, Schwarz A, Heimburger N. Determination of human thrombin-antithrombin III complex in plasma with an enzymelinked immunosorbent assay. Thromb Haemost 59: 101, 1988. Elms MJ, Bunce IH, Bundesen PG, et al. Measurement of crosslinked fibrin degradation products - animmunoassay using monoclonal antibodies. Thromb Haemost 50: 591, 1983. Kawakami M, Kawagoe M, Harigai M, et al. Elevated plasma
Internal
Medicine
Vol. 31, No. 12 (December
1992)
levels of a^-plasmin inhibitor-plasmin complex in patients with rheumatic disease. Possible role of fibrinolytic mechanism in vasculitis. Arthritis Rheum 32: 1427, 1989. Sudo T, Sawai M. Immunoreactivities of fibrin (ogen) related antigens and antibodies in radioimmunoassay and latex photo metric immunoassay. Acta Haematol Jpn 44: 131, 1981. Feinstein DI, Rapaport SI. Acquired inhibitors of blood coagu lation. Prog Hemost Thromb 1: 75, 1972. Feltkamp T, Kirkwood T, Maini R, Aarden L. The first inter national standard for antibodies to double stranded DNA. Ann Rheum Dis 47: 740, 1988. Urowitz MB, Gladman DD, Tozman ECS, Goldsmith CH. The lupus activity criteria count (LACC). J Rheumatol ll: 783, 1984. Gurian LE, Rogoff TM, Ware AJ, et al. The immunologic diagnosis of chronic active "autoimmune" hepatitis: Distinction from systemic lupus erythematosus. Hepatology 5: 397, 1985. Decker JL, Steinberg AD, Gershwin ME, et al. Systemic lupus erythematosus - Contrasts and comparisons. Ann Intern Med 82: 391, 1975. Rothfield NF. Systemic lupus erythematosus. in: Rheumatic Diseases: Diagnosis and Management, Katz WA, Ed. Philadelphia, JB Lipincott Co, p765, 1977. Runyon BA, LaBrecque DR, Anuras S. The spectrum of liver disease in systemic lupus erythematosus. Report of 33 histologically proved cases and review of the literature. Am J Med 69: 187, 1980.
1395
