Abstract
Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catastrophic antiphospholipid syndrome (CAPS) is a rare and often fatal form of APS characterized by disseminated intravascular thrombosis and ischemic injury resulting in multiorgan failure. Rarely, intravascular thrombosis in CAPS is accompanied by hemorrhagic manifestations such as diffuse alveolar hemorrhage. Here, we report a 43-year-old woman who presented with anemia, acute gastroenteritis, abnormal liver function tests, bilateral pulmonary infiltrates, and a systemic inflammatory response syndrome. The patient developed respiratory failure as a result of diffuse alveolar hemorrhage followed by acute renal failure. Laboratory tests disclosed hematuria, proteinuria, and reduced platelet count. Microbiologic tests were negative. A renal biopsy demonstrated acute thrombotic microangiopathy and extensive interstitial hemorrhage. Serologic tests disclosed antinuclear antibodies and reduced serum complement C4 concentration. Coagulation studies revealed the lupus anticoagulant and autoantibodies against cardiolipin, beta 2-glycoprotein I, and prothrombin. High-dose glucocorticoids and plasma exchange resulted in rapid resolution of pulmonary, renal, and hematological manifestations. This rare case emphasizes that CAPS can present with concurrent thrombotic and hemorrhagic manifestations. Rapid diagnosis and treatment may result in complete recovery.
| Original language | English |
|---|---|
| Pages (from-to) | 855-864 |
| Number of pages | 10 |
| Journal | Lupus |
| Volume | 22 |
| Issue number | 8 |
| DOIs | |
| State | Published - Jul 1 2013 |
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Keywords
- CAPS
- Catastrophic antiphospholipid syndrome
- diffuse alveolar hemorrhage
- glucocorticoids
- hemorrhage
- interstitial hemorrhage
- plasma exchange
- plasmapheresis
- thrombotic microangiopathy
ASJC Scopus subject areas
- Rheumatology
Cite this
Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations. / Rangel, M. L.; Alghamdi, I.; Contreras, Gabriel; Harrington, Thomas; Thomas, David; Barisoni-Thomas, Laura; Andrews, David; Wolf, M.; Asif, A.; Nayer, Ali.
In: Lupus, Vol. 22, No. 8, 01.07.2013, p. 855-864.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Catastrophic antiphospholipid syndrome with concurrent thrombotic and hemorrhagic manifestations
AU - Rangel, M. L.
AU - Alghamdi, I.
AU - Contreras, Gabriel
AU - Harrington, Thomas
AU - Thomas, David
AU - Barisoni-Thomas, Laura
AU - Andrews, David
AU - Wolf, M.
AU - Asif, A.
AU - Nayer, Ali
PY - 2013/7/1
Y1 - 2013/7/1
N2 - Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catastrophic antiphospholipid syndrome (CAPS) is a rare and often fatal form of APS characterized by disseminated intravascular thrombosis and ischemic injury resulting in multiorgan failure. Rarely, intravascular thrombosis in CAPS is accompanied by hemorrhagic manifestations such as diffuse alveolar hemorrhage. Here, we report a 43-year-old woman who presented with anemia, acute gastroenteritis, abnormal liver function tests, bilateral pulmonary infiltrates, and a systemic inflammatory response syndrome. The patient developed respiratory failure as a result of diffuse alveolar hemorrhage followed by acute renal failure. Laboratory tests disclosed hematuria, proteinuria, and reduced platelet count. Microbiologic tests were negative. A renal biopsy demonstrated acute thrombotic microangiopathy and extensive interstitial hemorrhage. Serologic tests disclosed antinuclear antibodies and reduced serum complement C4 concentration. Coagulation studies revealed the lupus anticoagulant and autoantibodies against cardiolipin, beta 2-glycoprotein I, and prothrombin. High-dose glucocorticoids and plasma exchange resulted in rapid resolution of pulmonary, renal, and hematological manifestations. This rare case emphasizes that CAPS can present with concurrent thrombotic and hemorrhagic manifestations. Rapid diagnosis and treatment may result in complete recovery.
AB - Antiphospholipid syndrome (APS) is a distinct autoimmune prothrombotic disorder due to pathogenic autoantibodies directed against proteins that bind to phospholipids. APS is characterized by arterial and venous thrombosis and their clinical sequelae. Catastrophic antiphospholipid syndrome (CAPS) is a rare and often fatal form of APS characterized by disseminated intravascular thrombosis and ischemic injury resulting in multiorgan failure. Rarely, intravascular thrombosis in CAPS is accompanied by hemorrhagic manifestations such as diffuse alveolar hemorrhage. Here, we report a 43-year-old woman who presented with anemia, acute gastroenteritis, abnormal liver function tests, bilateral pulmonary infiltrates, and a systemic inflammatory response syndrome. The patient developed respiratory failure as a result of diffuse alveolar hemorrhage followed by acute renal failure. Laboratory tests disclosed hematuria, proteinuria, and reduced platelet count. Microbiologic tests were negative. A renal biopsy demonstrated acute thrombotic microangiopathy and extensive interstitial hemorrhage. Serologic tests disclosed antinuclear antibodies and reduced serum complement C4 concentration. Coagulation studies revealed the lupus anticoagulant and autoantibodies against cardiolipin, beta 2-glycoprotein I, and prothrombin. High-dose glucocorticoids and plasma exchange resulted in rapid resolution of pulmonary, renal, and hematological manifestations. This rare case emphasizes that CAPS can present with concurrent thrombotic and hemorrhagic manifestations. Rapid diagnosis and treatment may result in complete recovery.
KW - CAPS
KW - Catastrophic antiphospholipid syndrome
KW - diffuse alveolar hemorrhage
KW - glucocorticoids
KW - hemorrhage
KW - interstitial hemorrhage
KW - plasma exchange
KW - plasmapheresis
KW - thrombotic microangiopathy
UR - http://www.scopus.com/inward/record.url?scp=84879733240&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84879733240&partnerID=8YFLogxK
U2 - 10.1177/0961203313491024
DO - 10.1177/0961203313491024
M3 - Article
C2 - 23722230
AN - SCOPUS:84879733240
VL - 22
SP - 855
EP - 864
JO - Lupus
JF - Lupus
SN - 0961-2033
IS - 8
ER -