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LETTER TO EDITOR
Ahead of print publication  

Catastrophic Antiphospholipid Syndrome Presenting As Ischemic Colitis


 Department of Internal Medicine, Nizar Hospital, Malappuram, Kerala, India

Date of Submission10-Dec-2021
Date of Acceptance15-Apr-2022
Date of Web Publication06-Jul-2022

Correspondence Address:
Mansoor C Abdulla,
Department of Internal Medicine, Nizar Hospital, Pattambi Road, Valancheri, Malappuram - 676 552, Kerala
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_274_21



How to cite this URL:
Abdulla MC. Catastrophic Antiphospholipid Syndrome Presenting As Ischemic Colitis. Indian J Rheumatol [Epub ahead of print] [cited 2022 Aug 14]. Available from: https://www.indianjrheumatol.com/preprintarticle.asp?id=350021



Dear Editor,

Catastrophic thrombotic syndromes present as sudden onset thromboembolic occlusions affecting different vascular beds. The diseases which can have such presentations include catastrophic antiphospholipid syndrome (CAPS), atypical thrombotic thrombocytopenic purpura, heparin-induced thrombocytopenia, and Trousseau syndrome. CAPS is a lethal complication of antiphospholipid syndrome (APS) which causes multiorgan failure secondary to disseminated vascular thrombosis.

A 37-year-old female presented to the surgery department with acute onset abdominal pain, vomiting, and constipation for 2 days. The abdominal pain was acute in onset, diffuse, intermittent with no radiation, or relation to food intake. She had multiple episodes of vomiting which was nonprojectile, bilious, and not bloodstained. The abdomen was not distended, with diffuse tenderness, but there was no guarding or rigidity. There was no hepatosplenomegaly or shifting dullness, and bowel sounds were decreased. Two days after the admission, she developed acute onset breathlessness. The examination then showed tachycardia and tachypnea with normal blood pressure. Two years back, she was diagnosed to have primary APS (when evaluated for multiple spontaneous first and second trimester abortions) and was started on warfarin.

Hemoglobin was 8.9 g/dl, leukocyte count – 8500/ml, platelet count – 19,000/microliter, and C-reactive protein was high. In the peripheral smear, red blood cells were normocytic with schistocytes, white blood cells were normal, and platelet count was markedly decreased suggesting microangiopathic hemolysis [Figure 1]a. Urinalysis showed 2+ albuminuria with 10–15 red blood cells per high-power field. Twenty-four urine protein was 1900 mg. Serum creatinine was 1.4 mg/dl, and blood urea was 44 mg/dl. The international normalized ratio was 2.4, and partial thromboplastin time was 72 s. She had markedly elevated D-dimer 6590 ng/mL (normal range 220–500 ng/mL).
Figure 1: Peripheral smear showing schistocytes suggesting microangiopathic hemolysis (a). Contrast-enhanced computed tomogram of the abdomen and thorax showing a thin-walled dilated colon (6.08cms), bowel wall thickening, left-sided pulmonary infarction, and uniform thickening of the abdominal aorta (b-d)

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Ultrasonography of the abdomen showed hyperechoic renal parenchyma. Contrast-enhanced computed tomogram of the abdomen showed a thin-walled dilated colon (6.08 cm), bowel wall thickening, and uniform thickening of the abdominal aorta [Figure 1]b, [Figure 1]c, [Figure 1]d. Contrast-enhanced computed tomogram of the thorax showed left-sided pulmonary infarction. Colonoscopy was normal. Two-dimensional echocardiogram was normal.

Immunoglobulin G (IgG) and immunoglobulin M (IgM) anti-cardiolipin antibody and lupus anticoagulant were positive, but IgG and IgM beta-2 glycoprotein were negative. An antinuclear antibody and anti-double-stranded DNA were negative. C3 and C4 levels were normal. Blood culture and COVID-19 nasal swab reverse transcriptase–polymerase were negative. The investigations of this 37-year-old female suggested the presence of a diffuse catastrophic thrombotic syndrome (microangiopathic hemolysis in peripheral smear, abdominal imaging showing colonic ischemia, pulmonary infarction, glomerulonephritis, and markedly elevated D-dimer), which was considered to be CAPS in the presence of positive antiphospholipid antibodies.

She was started on methylprednisolone 1000 mg intravenously daily (given for 5 days), enoxaparin 60 mg twice daily, and was kept nil per oral. She had a significant improvement in her symptoms after 5 days of treatment. The hemoglobin and platelet count also improved. She was started on oral prednisolone 1 mg/kg later which was tapered and enoxaparin was continued.

Catastrophic thrombotic syndromes can be seen in individuals with or without a preexisting prothrombotic disorder.[1] CAPS can evolve rapidly causing multiple vascular occlusive events involving microvasculature of various internal organs (brain, lungs, and kidneys).[1]

The diagnosis of CAPS can be definitive if all the following four criteria are met. 1) Involvement of three or more organs/tissues, 2) Development of manifestations in less than a week, 3) Histological evidence of intravascular thrombosis, 4) Presence of antiphospholipid antibodies on two occasions six weeks apart. The diagnosis of CAPS is probable when three criteria are complete with an incomplete fourth.[2] Our patient developed a catastrophic disease involving the intestine, lungs, and kidneys in less than a week. We were not able to establish a histological diagnosis of vascular thrombosis due to severe thrombocytopenia. However, the presence of microangiopathic hemolytic anemia and markedly elevated D-dimer indicate a thrombotic process.

Respiratory system involvement in CAPS can cause pulmonary embolism and infarction, pulmonary hypertension, adult respiratory distress syndrome, alveolar hemorrhage, primary thrombosis of lung vessels, and pulmonary capillaritis.[3] Renal manifestations of CAPS occur in 71% of patients, and the most common finding histologically is acute thrombotic microangiopathy.[4] Hypertension, proteinuria, hematuria, and acute renal failure are the common manifestations of renal disease in CAPS. Gastrointestinal involvement, such as Budd–Chiari syndrome, pancreatitis, hepatic or splenic thrombosis, bowel necrosis, and ischemic colitis, has been previously reported.[5],[6],[7] The mortality rate of CAPS is nearly 50% and it accounts for <1% of all cases of APS. Treatment (intravenous heparin, high doses of intravenous steroids, immunoglobulins, and plasmapheresis) can reduce mortality from 50% to 30%.[8] The possible reason for the thickening of the aortic wall on imaging in our patient was considered to be chronic periaortitis. Chronic periaortitis is a rare idiopathic disease, characterized by thickening of the aortic wall, which may vary from an inflammatory infiltrate in the acute phase until a fibrous scar in more advanced stages. This can be associated with antiphospholipid syndrome.[9]

To conclude, early recognition of catastrophic thrombotic syndromes such as CAPS is essential for proper management, which can reduce mortality. Surgeons and physicians should be aware of the diverse presentations of CAPS affecting diverse vascular beds. The presence of microangiopathic hemolytic anemia and elevated markers of thrombosis such as D-dimer along with thromboembolic occlusions affecting different organs should prompt one to think of CAPS even when a histological diagnosis of vascular thrombosis cannot be established.

Ethics approval

All procedures performed in studies involving human participants were by the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that their name and initials will not be published, and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ortel TL, Erkan D, Kitchens CS. How I treat catastrophic thrombotic syndromes. Blood 2015;126:1285-93.  Back to cited text no. 1
    
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. 2
    
3.
Espinosa G, Cervera R, Font J, Asherson RA. The lung in the antiphospholipid syndrome. Ann Rheum Dis 2002;61:195-8.  Back to cited text no. 3
    
4.
Cervera R, Bucciarelli S, Plasín MA, Gómez-Puerta JA, Plaza J, Pons-Estel G, et al. Catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun 2009;32:240-5.  Back to cited text no. 4
    
5.
González Puga C, Lendínez Romero I, Palomeque Jiménez A. Catastrophic antiphospholipid antibody syndrome presenting as severe ischaemic colitis. Gastroenterol Hepatol 2017;40:684-7.  Back to cited text no. 5
    
6.
Jürgensen JS, Kettritz R, Schneider W, Koop H, Hildebrand TS, Frei U, et al. Catastrophic antiphospholipid syndrome masquerading as ischaemic colitis. Rheumatol Int 2003;23:204-6.  Back to cited text no. 6
    
7.
Ruffatti A, Calligaro A, Lacognata CS, D'Odorico A, Colpo A, Cardin F, et al. Insights into the pathogenesis of catastrophic antiphospholipid syndrome. A case report of relapsing catastrophic antiphospholipid syndrome and review of the literature on ischemic colitis. Clin Rheumatol 2020;39:1347-55.  Back to cited text no. 7
    
8.
Sciascia S, Lopez-Pedrera C, Roccatello D, Cuadrado MJ. Catastrophic antiphospholipid syndrome (CAPS). Best Pract Res Clin Rheumatol 2012;26:535-41.  Back to cited text no. 8
    
9.
Carneiro L, Ferreira A, Rios E. Chronic periaortitis and antiphospholipid syndrome: Is there a link? Galicia Clín 2016;77:71-4.  Back to cited text no. 9
    


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