Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Print this page Email this page Users Online: 6503

  Table of Contents  
Year : 2022  |  Volume : 15  |  Issue : 1  |  Page : 118-120  

Double trouble - Catastrophic antiphospholipid antibody syndrome with dengue fever

1 Department of Pulmonary Medicine, Yashoda Hospital, Kaushambi, India
2 Department of Pulmonary Medicine, Aanch Hospital, Jaipur, Rajasthan, India
3 Chairman, Metro Centre for Respiratory Diseases, Metro Hospital, Noida, UP, India

Date of Submission24-Jul-2020
Date of Decision15-Sep-2020
Date of Acceptance25-Sep-2020
Date of Web Publication19-May-2021

Correspondence Address:
Arjun Khanna
Yashoda Superspeciality Hospital, Kaushambi, Uttar Pradesh
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_408_20

Rights and Permissions

The clinical spectrum of dengue fever is ever increasing as we encounter more cases of this disease regularly for the past few years. Dengue fever continues to surprise us with its myriad presentations and combinations with other disease entities. Catastrophic antiphospholipid antibody syndrome (APS) is uncommon and often a fatal form of primary APS. Here, we present a 37-year-old lady who presented with catastrophic APS with dengue fever. The coexistence of these two conditions has not been reported earlier, to the best of our knowledge.

Keywords: Antiphospholipid antibody syndrome, Dengue fever, Multi organ dysfunction

How to cite this article:
Khanna A, Periwal P, Talwar D. Double trouble - Catastrophic antiphospholipid antibody syndrome with dengue fever. Med J DY Patil Vidyapeeth 2022;15:118-20

How to cite this URL:
Khanna A, Periwal P, Talwar D. Double trouble - Catastrophic antiphospholipid antibody syndrome with dengue fever. Med J DY Patil Vidyapeeth [serial online] 2022 [cited 2022 Aug 11];15:118-20. Available from: https://www.mjdrdypv.org/text.asp?2022/15/1/118/316424

  Introduction Top

Catastrophic antiphospholipid antibody syndrome (APS) is an unusual form of presentation that represents <1% of the APS cases.[1] Patients mostly present as life-threatening emergencies with mortality rates as high as 50%. The most important distinguishing feature is the involvement of small vessel in catastrophic APS. Our patient was a 37-year-old woman who presented with fever and thrombocytopenia and was diagnosed to be having dengue fever. Despite appropriate management of the dengue fever, her condition worsened and a diagnosis of catastrophic APS was made based on the clinical and laboratory parameters.

  Case Report Top

A 37-year-old woman presented to the emergency room with the complaints of high-grade fever, generalized body ache, myalgia, and abdominal pain with multiple episodes of nonbilious vomiting for the last 3–4 days. On presentation, she was severely dehydrated with a heart rate of 124/min, respiratory rate of 28/min, temperature 100°F, blood pressure of 80/50 mmHg, and saturation 96% on room air. General examination revealed cold peripheries and dehydrated skin and mucus membranes. Systemic examination was grossly normal.

Her laboratory parameters were hemoglobin of 7.3 g/dl, total leukocyte count of 16,800 cumm, platelet count of 84,000 cumm, serum urea of 17 mg/dl, serum creatinine of 0.80 mg/dl, serum sodium of 129 mmol/dl, serum potassium of 3.2 mmol/dl, total bilirubin of 0.6 mg/dl, direct bilirubin of 0.2 mg/dl, aspartate aminotransferase of 34 IU/L, and alanine aminotransferase of 42 IU/L. Prothrombin time and international standardized ratio (INR) were deranged 42.5/3.6. Serum amylase and lipase were within normal limits. Blood smear and antigen detection test for malaria were negative. In view of clustering of dengue cases and her complains, dengue serology was sent, and she tested positive for nonstructural protein 1 dengue antigen. She was initiated on treatment with intravenous fluids, analgesics, antiemetics, and antacids, and two units of packed red blood cells was transfused. Ultrasound of thewhole abdomen showed hepatomegaly (18.9 cm), distended gallbladder with mildly thick wall. Chest X-ray showed bilateral minimal pleural effusion.

Over the next 2 days, she developed worsening thrombocytopenia (platelet count 5000/cumm) and was transfused multiple units of platelets. Despite continuous infusion of intravenous proton pump inhibitors and antiemetics, her pain abdomen and vomiting worsened. A gastroenterology opinion was taken, and contrast-enhanced computerized tomogram (CECT) of the abdomen was advised. CECT of the abdomen showed that the liver was enlarged with multiple small hypodense lesions in the left and right lobes, with focal areas of hyperdensity in both lobes and multiple retroperitoneal lymph nodes. The hepatic lesions were labeled as either developing cholangiolar abscesses or liver infarcts. Right-sided  Fallopian tube More Details was not visualized properly on the computed tomography (CT) scan and its anatomy was distorted, and it appeared as a complex tubo-ovarian mass, which could not be properly characterized on the CT scan. Note was made of an inferior vena cava filter.

On probing the history, the patient revealed that she had primary infertility with bad obstetric history and multiple first-trimester spontaneous abortions and had undergone multiple cycles of in vitro fertilization, 10 years ago, one of which lead to an ectopic pregnancy in the right fallopian tube, which was then surgically repaired. Postsurgery, during the hospital stay, the patient developed swelling in the left lower limb, after which she was started on oral medications, of which she had no records. As per the patient, despite being on medications, the patient had developed similar swelling in her right lower limb, after which some procedure was done and a stent was placed in her abdomen. After the procedure, she took oral medications for 2–3 months and discontinued all treatment.

She continued to have worsening pain abdomen and vomiting, and owing to her history of thrombophilia, a CT angiogram of the abdominal vessels was asked for, to rule out mesenteric ischemia. CT angiogram of the abdomen did not reveal any bowel wall ischemia. Extension of the contrast into the other vessels surprisingly revealed extensive superior vena caval thrombosis. Over the next few days, she developed worsening thrombocytopenia, bilateral pleural effusions and ascites, and hypotension and had to be given vasopressors and multiple blood products, including packed red blood cells and platelets.

Worsening thrombocytopenia and pleural effusions were attributed to the dengue fever. However, her pain abdomen and abdominal radiological findings could not be explained by dengue fever.

Recurrent history of thrombophilia along with thrombocytopenia led to a suspicion of APS. A detailed profile was sent to evaluate for APS. Serum antiphospholipid immunoglobulin G (IgG) antibody was raised to more than 100 GPL units/ml (normal: 0–10 GPL units/ml), Serum Antiphospholipid IgA antibody was 19 U/ml (normal <12 U/ml), serum antiphospholipid IgM antibody was 2.2 U/ml (<5 U/ml), and lupus anticoagulant and serum beta 2 glycoprotein IgM were negative.

Despite blood products and other supportive measures, she continued have worsening anemia and thrombocytopenia and have developed multiorgan dysfunction with hepatic and renal derangement. A diagnosis of catastrophic APS secondary to dengue fever was made, and the patient was given pulse steroid therapy with 1 g methyl prednisolone for 3 days and subcutaneous low-molecular-weight heparin, under the cover of blood products. Over the next few days, the patient made gradual recovery and improved clinically. The multiorgan dysfunction reverted back to normal. The patient was shifted to oral steroid and dabigatran 110 mg twice daily and discharged from the hospital.

To the best of our knowledge, this is the first reported case of catastrophic APS secondary to dengue fever.

  Discussion Top

APS is an autoimmune disease characterized by the presence of thromboembolic complications, bad obstetric history, and multiorgan dysfunction, in the presence of persistently increased titers of antiphospholipid antibodies (APLAs). The most commonly detected subgroups of APLA are lupus anticoagulant, anticardiolipin, and anti-beta-2-glycoprotein 1 antibodies.[2] Catastrophic APS is an exaggerated form of APS, resulting in multiorgan failure and a high mortality rate.[3] The diagnosis of catastrophic APS is based on the following criteria: (1) evidence of involvement of 3 or more organs, systems, and/or tissues; (2) development of manifestations simultaneously or in less than a week; (3) confirmation by histopathology of small-vessel occlusion in at least 1 organ or tissue; (4) laboratory confirmation of the presence of APLA (lupus anticoagulant and/or anticardiolipin antibodies).[4] If all four criteria are met, the patient falls into the definite catastrophic APS; otherwise, the patient falls under the probable catastrophic APS.[5] We could not do liver biopsy in our patient, owing to the high risk of bleeding in our patient. Most cases have a precipitating event in the form of an infection or surgery. In our patient, the dengue fever could have been a possible inciting event. Our patient had more than three organs involved in the form of liver infarcts (secondary to microthrombotic occlusion, not visible on imaging), hematological involvement, and renal dysfunction along with polyserositis and superior vena caval thrombosis. Management of catastrophic APS includes treatment of the precipitating factors, stopping the thrombotic process, removing aPL, and suppressing the excessive cytokine release.[4],[5] Corticosteroids, intravenous Ig, and plasmapheresis are used as a part of standard treatment, while rituximab has been used in cases resistant to standard care.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Erkan D, Cervera R, Asherson RA. Catastrophic antiphospholipid syndrome: Where do we stand? Arthritis Rheum 2003;48:3320-7.  Back to cited text no. 1
Devreese K, Peerlinck K, Hoylaerts MF. Thrombotic risk assessment in the antiphospholipid syndrome requires more than the quantification of lupus anticoagulants. Blood 2010;115:870-78.  Back to cited text no. 2
Cervera R, Font J, Gómez-Puerta JA, Espinosa G, Cucho M, Bucciarelli S, et al. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005;64:1205-9.  Back to cited text no. 3
Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, et al. Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus 2003;12:530-4.  Back to cited text no. 4
Cervera R; CAPS Registry Project Group. Catastrophic antiphospholipid syndrome (CAPS): Update from the 'CAPS Registry.' Lupus. 2010;19:412-8.  Back to cited text no. 5


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
  Case Report

 Article Access Statistics
    PDF Downloaded41    
    Comments [Add]    

Recommend this journal