Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus

A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepati...

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Veröffentlicht in:Acta medica Indonesiana 2018-10, Vol.50 (4), p.343-345
Hauptverfasser: Darmawan, Guntur, Hamijoyo, Laniyati, Oehadian, Amaylia, Bandiara, Ria, Amalia, Lisda
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Hamijoyo, Laniyati
Oehadian, Amaylia
Bandiara, Ria
Amalia, Lisda
description A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Her obstetrical history was marked by miscarriage in second pregnancy and preeclampsia in third pregnancy. She used hormonal contraceptives until 5 months prior to admission. On physical examination, she had anemic conjunctiva and no organomegaly. Blood tests were significant for anemia (3.4 g/dl) and thrombocytopenia (28,000/µl). Her vaginal bleeding had ceased, however her platelet continued decreasing to 12,000/µl during first several days of hospitalization despite receiving platelet transfusion. On the tenth hospital day, she suddenly complained of severe headache and blurred vision. She had bilateral edema and erythema of palpebral, chemosis, decreased in visual acuity, and reduced ocular motility. Ear and nose examination were normal. Peripheral blood smear showed no blast. Prothrombine time (PT), INR, APTT tests were normal and D Dimer was slightly increased (3.3 mg/l; NV ≤0.5 mg/l). Urine examination revealed proteinuria with 24 hour urine protein was 1,863 mg (NV
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One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Her obstetrical history was marked by miscarriage in second pregnancy and preeclampsia in third pregnancy. She used hormonal contraceptives until 5 months prior to admission. On physical examination, she had anemic conjunctiva and no organomegaly. Blood tests were significant for anemia (3.4 g/dl) and thrombocytopenia (28,000/µl). Her vaginal bleeding had ceased, however her platelet continued decreasing to 12,000/µl during first several days of hospitalization despite receiving platelet transfusion. On the tenth hospital day, she suddenly complained of severe headache and blurred vision. She had bilateral edema and erythema of palpebral, chemosis, decreased in visual acuity, and reduced ocular motility. Ear and nose examination were normal. Peripheral blood smear showed no blast. Prothrombine time (PT), INR, APTT tests were normal and D Dimer was slightly increased (3.3 mg/l; NV ≤0.5 mg/l). Urine examination revealed proteinuria with 24 hour urine protein was 1,863 mg (NV &lt;150 mg/day). We assessed her as cavernous sinus thrombosis and treated her empirically with intravenous broad-spectrum antibiotics, morphine drip. Either digital subtraction angiography or anticoagulant was deferred due to low platelet. Further examination revealed positive for ANA, anti-SSA, and diagnosis of SLE was established. Anticardiolipin antibodies of IgG and IgM and anti-beta2 glycoprotein antibodies of IgM and IgG tests were non reactive. Methylprednisolone pulse therapy (1g/day) was given for 3 consecutive days, and then tapered to oral methylprednisolone. She additionally received azathioprine 50 mg tab BID. Meanwhile her clinical symptoms alleviated and platelet count was increased, brain MRI and MR venography finally performed suggesting cerebral venous sinus thrombosis. She got additional oral anticoagulant rivaroxaban 15 mg tab BID and eventually discharged. Cerebral venous sinus thrombosis may be the presenting symptoms or occur concomitantly within the onset of SLE. Our patient had SLE, meeting 4 of the Systemic Lupus International Collaborating Clinic classification criteria (hemolytic anemia, thrombocytopenia, renal involvement, and positive for ANA test). Vasculitis due to endothelial cell injury mediated by immune-complex deposition is proposed to be the pathogenesis of CVST in SLE. Hypercoagulable state could be other etiology factor. Antiphospholipid antibodies were absent in our case as reported in some cases, emphasizing vasculitis as the underlying mechanism. Treatment of CVST in SLE consisting of anticoagulant, steroid, and immunosuppressant. This case elicits intriguing problem: CVST and thrombocytopenia. Anticoagulant treatment is proposed as the cornerstone treatment for CVST, however it was deferred due to risk of bleeding in thrombocytopenia. Steroid plays role in treatment of CVST in SLE, owing to its anti-inflammatory property. As shown in previous cases, the patient had remarkable response to high dose steroid treatment and eventually got anticoagulant after her platelet had increased. In summary, prompt diagnosis and treatment of CVST are important for a favorable prognosis.</description><identifier>ISSN: 0125-9326</identifier><identifier>PMID: 30631001</identifier><language>eng</language><publisher>Indonesia</publisher><subject>Adult ; Anemia, Hemolytic, Autoimmune - complications ; Anemia, Hemolytic, Autoimmune - drug therapy ; Anticoagulants - therapeutic use ; beta 2-Glycoprotein I - therapeutic use ; Female ; Humans ; Immunosuppressive Agents - therapeutic use ; Lupus Erythematosus, Systemic - complications ; Lupus Erythematosus, Systemic - diagnosis ; Lupus Erythematosus, Systemic - drug therapy ; Magnetic Resonance Imaging ; Methylprednisolone - therapeutic use ; Sinus Thrombosis, Intracranial - diagnostic imaging ; Sinus Thrombosis, Intracranial - drug therapy ; Thrombocytopenia - complications ; Thrombocytopenia - drug therapy</subject><ispartof>Acta medica Indonesiana, 2018-10, Vol.50 (4), p.343-345</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30631001$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Darmawan, Guntur</creatorcontrib><creatorcontrib>Hamijoyo, Laniyati</creatorcontrib><creatorcontrib>Oehadian, Amaylia</creatorcontrib><creatorcontrib>Bandiara, Ria</creatorcontrib><creatorcontrib>Amalia, Lisda</creatorcontrib><title>Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus</title><title>Acta medica Indonesiana</title><addtitle>Acta Med Indones</addtitle><description>A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Her obstetrical history was marked by miscarriage in second pregnancy and preeclampsia in third pregnancy. She used hormonal contraceptives until 5 months prior to admission. On physical examination, she had anemic conjunctiva and no organomegaly. Blood tests were significant for anemia (3.4 g/dl) and thrombocytopenia (28,000/µl). Her vaginal bleeding had ceased, however her platelet continued decreasing to 12,000/µl during first several days of hospitalization despite receiving platelet transfusion. On the tenth hospital day, she suddenly complained of severe headache and blurred vision. She had bilateral edema and erythema of palpebral, chemosis, decreased in visual acuity, and reduced ocular motility. Ear and nose examination were normal. Peripheral blood smear showed no blast. Prothrombine time (PT), INR, APTT tests were normal and D Dimer was slightly increased (3.3 mg/l; NV ≤0.5 mg/l). Urine examination revealed proteinuria with 24 hour urine protein was 1,863 mg (NV &lt;150 mg/day). We assessed her as cavernous sinus thrombosis and treated her empirically with intravenous broad-spectrum antibiotics, morphine drip. Either digital subtraction angiography or anticoagulant was deferred due to low platelet. Further examination revealed positive for ANA, anti-SSA, and diagnosis of SLE was established. Anticardiolipin antibodies of IgG and IgM and anti-beta2 glycoprotein antibodies of IgM and IgG tests were non reactive. Methylprednisolone pulse therapy (1g/day) was given for 3 consecutive days, and then tapered to oral methylprednisolone. She additionally received azathioprine 50 mg tab BID. Meanwhile her clinical symptoms alleviated and platelet count was increased, brain MRI and MR venography finally performed suggesting cerebral venous sinus thrombosis. She got additional oral anticoagulant rivaroxaban 15 mg tab BID and eventually discharged. Cerebral venous sinus thrombosis may be the presenting symptoms or occur concomitantly within the onset of SLE. Our patient had SLE, meeting 4 of the Systemic Lupus International Collaborating Clinic classification criteria (hemolytic anemia, thrombocytopenia, renal involvement, and positive for ANA test). Vasculitis due to endothelial cell injury mediated by immune-complex deposition is proposed to be the pathogenesis of CVST in SLE. Hypercoagulable state could be other etiology factor. Antiphospholipid antibodies were absent in our case as reported in some cases, emphasizing vasculitis as the underlying mechanism. Treatment of CVST in SLE consisting of anticoagulant, steroid, and immunosuppressant. This case elicits intriguing problem: CVST and thrombocytopenia. Anticoagulant treatment is proposed as the cornerstone treatment for CVST, however it was deferred due to risk of bleeding in thrombocytopenia. Steroid plays role in treatment of CVST in SLE, owing to its anti-inflammatory property. As shown in previous cases, the patient had remarkable response to high dose steroid treatment and eventually got anticoagulant after her platelet had increased. In summary, prompt diagnosis and treatment of CVST are important for a favorable prognosis.</description><subject>Adult</subject><subject>Anemia, Hemolytic, Autoimmune - complications</subject><subject>Anemia, Hemolytic, Autoimmune - drug therapy</subject><subject>Anticoagulants - therapeutic use</subject><subject>beta 2-Glycoprotein I - therapeutic use</subject><subject>Female</subject><subject>Humans</subject><subject>Immunosuppressive Agents - therapeutic use</subject><subject>Lupus Erythematosus, Systemic - complications</subject><subject>Lupus Erythematosus, Systemic - diagnosis</subject><subject>Lupus Erythematosus, Systemic - drug therapy</subject><subject>Magnetic Resonance Imaging</subject><subject>Methylprednisolone - therapeutic use</subject><subject>Sinus Thrombosis, Intracranial - diagnostic imaging</subject><subject>Sinus Thrombosis, Intracranial - drug therapy</subject><subject>Thrombocytopenia - complications</subject><subject>Thrombocytopenia - drug therapy</subject><issn>0125-9326</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo1T81qwzAY82FjLV1fYeS4S8Cxa8e-bYTuBwo7tNs1fHE-rx5xktnJIW8_w9qLBJIQ0g1Z04KJXHMmV2Qb4w-ltKCcq1LekRWnkhdJWJOnCgM2AbrsC_thjtnR9QlP5zD4ZoguZq7Pjkuc0DuTHeYxmfuwTGf0MA1xjvfk1kIXcXvhDfl82Z-qt_zw8fpePR_yb8bLKZdCCbOzAgzjxqIVO9uWrdKaNVy2wCxiowRaXYI2AKCwVdSUwIxh0tiCb8jjf-8Yht8Z41R7Fw12HfSYdtesKDXn6TRL0YdLdG48tvUYnIew1NfX_A_EkFSC</recordid><startdate>20181001</startdate><enddate>20181001</enddate><creator>Darmawan, Guntur</creator><creator>Hamijoyo, Laniyati</creator><creator>Oehadian, Amaylia</creator><creator>Bandiara, Ria</creator><creator>Amalia, Lisda</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>20181001</creationdate><title>Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus</title><author>Darmawan, Guntur ; Hamijoyo, Laniyati ; Oehadian, Amaylia ; Bandiara, Ria ; Amalia, Lisda</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-g237t-6585c4f5ac23cfef54fd7d8992b36da2feeb85ef97a9caaa8ed80c7a2cc26cf13</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Adult</topic><topic>Anemia, Hemolytic, Autoimmune - complications</topic><topic>Anemia, Hemolytic, Autoimmune - drug therapy</topic><topic>Anticoagulants - therapeutic use</topic><topic>beta 2-Glycoprotein I - therapeutic use</topic><topic>Female</topic><topic>Humans</topic><topic>Immunosuppressive Agents - therapeutic use</topic><topic>Lupus Erythematosus, Systemic - complications</topic><topic>Lupus Erythematosus, Systemic - diagnosis</topic><topic>Lupus Erythematosus, Systemic - drug therapy</topic><topic>Magnetic Resonance Imaging</topic><topic>Methylprednisolone - therapeutic use</topic><topic>Sinus Thrombosis, Intracranial - diagnostic imaging</topic><topic>Sinus Thrombosis, Intracranial - drug therapy</topic><topic>Thrombocytopenia - complications</topic><topic>Thrombocytopenia - drug therapy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Darmawan, Guntur</creatorcontrib><creatorcontrib>Hamijoyo, Laniyati</creatorcontrib><creatorcontrib>Oehadian, Amaylia</creatorcontrib><creatorcontrib>Bandiara, Ria</creatorcontrib><creatorcontrib>Amalia, Lisda</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>Acta medica Indonesiana</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Darmawan, Guntur</au><au>Hamijoyo, Laniyati</au><au>Oehadian, Amaylia</au><au>Bandiara, Ria</au><au>Amalia, Lisda</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus</atitle><jtitle>Acta medica Indonesiana</jtitle><addtitle>Acta Med Indones</addtitle><date>2018-10-01</date><risdate>2018</risdate><volume>50</volume><issue>4</issue><spage>343</spage><epage>345</epage><pages>343-345</pages><issn>0125-9326</issn><abstract>A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Her obstetrical history was marked by miscarriage in second pregnancy and preeclampsia in third pregnancy. She used hormonal contraceptives until 5 months prior to admission. On physical examination, she had anemic conjunctiva and no organomegaly. Blood tests were significant for anemia (3.4 g/dl) and thrombocytopenia (28,000/µl). Her vaginal bleeding had ceased, however her platelet continued decreasing to 12,000/µl during first several days of hospitalization despite receiving platelet transfusion. On the tenth hospital day, she suddenly complained of severe headache and blurred vision. She had bilateral edema and erythema of palpebral, chemosis, decreased in visual acuity, and reduced ocular motility. Ear and nose examination were normal. Peripheral blood smear showed no blast. Prothrombine time (PT), INR, APTT tests were normal and D Dimer was slightly increased (3.3 mg/l; NV ≤0.5 mg/l). Urine examination revealed proteinuria with 24 hour urine protein was 1,863 mg (NV &lt;150 mg/day). We assessed her as cavernous sinus thrombosis and treated her empirically with intravenous broad-spectrum antibiotics, morphine drip. Either digital subtraction angiography or anticoagulant was deferred due to low platelet. Further examination revealed positive for ANA, anti-SSA, and diagnosis of SLE was established. Anticardiolipin antibodies of IgG and IgM and anti-beta2 glycoprotein antibodies of IgM and IgG tests were non reactive. Methylprednisolone pulse therapy (1g/day) was given for 3 consecutive days, and then tapered to oral methylprednisolone. She additionally received azathioprine 50 mg tab BID. Meanwhile her clinical symptoms alleviated and platelet count was increased, brain MRI and MR venography finally performed suggesting cerebral venous sinus thrombosis. She got additional oral anticoagulant rivaroxaban 15 mg tab BID and eventually discharged. Cerebral venous sinus thrombosis may be the presenting symptoms or occur concomitantly within the onset of SLE. Our patient had SLE, meeting 4 of the Systemic Lupus International Collaborating Clinic classification criteria (hemolytic anemia, thrombocytopenia, renal involvement, and positive for ANA test). Vasculitis due to endothelial cell injury mediated by immune-complex deposition is proposed to be the pathogenesis of CVST in SLE. Hypercoagulable state could be other etiology factor. Antiphospholipid antibodies were absent in our case as reported in some cases, emphasizing vasculitis as the underlying mechanism. Treatment of CVST in SLE consisting of anticoagulant, steroid, and immunosuppressant. This case elicits intriguing problem: CVST and thrombocytopenia. Anticoagulant treatment is proposed as the cornerstone treatment for CVST, however it was deferred due to risk of bleeding in thrombocytopenia. Steroid plays role in treatment of CVST in SLE, owing to its anti-inflammatory property. As shown in previous cases, the patient had remarkable response to high dose steroid treatment and eventually got anticoagulant after her platelet had increased. In summary, prompt diagnosis and treatment of CVST are important for a favorable prognosis.</abstract><cop>Indonesia</cop><pmid>30631001</pmid><tpages>3</tpages></addata></record>
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subjects Adult
Anemia, Hemolytic, Autoimmune - complications
Anemia, Hemolytic, Autoimmune - drug therapy
Anticoagulants - therapeutic use
beta 2-Glycoprotein I - therapeutic use
Female
Humans
Immunosuppressive Agents - therapeutic use
Lupus Erythematosus, Systemic - complications
Lupus Erythematosus, Systemic - diagnosis
Lupus Erythematosus, Systemic - drug therapy
Magnetic Resonance Imaging
Methylprednisolone - therapeutic use
Sinus Thrombosis, Intracranial - diagnostic imaging
Sinus Thrombosis, Intracranial - drug therapy
Thrombocytopenia - complications
Thrombocytopenia - drug therapy
title Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus
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