5 CARE-guideline compliant case reports · International journal publishing standard
BMJ Case Reports
ISSN 1757-790X · DOI: 10.1136/bcr-2024-259431
Received 14 August 2024
Accepted 22 October 2024
Published 12 November 2024
Priya Sharma¹, Ramesh Kumar Gupta¹, Sanjay Trivedi², Anita Bhatia³
¹ Department of General Medicine, AIIMS Bhopal, Bhopal 462020, India
² Department of Haematology, AIIMS Bhopal, Bhopal 462020, India
³ Department of Radiology, AIIMS Bhopal, Bhopal 462020, India
Correspondence: Dr. Ramesh Kumar Gupta, Department of General Medicine, AIIMS Bhopal. Email: rkgupta@aiimsbhopal.edu.in
Background: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening variant of antiphospholipid syndrome characterised by simultaneous multi-organ thrombosis within a short period. COVID-19 has been reported as a precipitating trigger, but cases from Indian populations remain extremely scarce.
Case presentation: A 28-year-old previously healthy Indian female presented with acute onset breathlessness, right lower limb pain and altered sensorium 10 days after SARS-CoV-2 infection (RT-PCR positive). Investigations revealed pulmonary embolism, deep vein thrombosis, cerebral venous sinus thrombosis and renal cortical necrosis occurring simultaneously. Triple-positive antiphospholipid antibodies were detected. CAPS was diagnosed using Asherson's criteria. She was treated with anticoagulation, intravenous immunoglobulins, methylprednisolone pulse therapy and plasma exchange with excellent clinical recovery.
Conclusions: COVID-19 can precipitate CAPS in predisposed individuals. Early recognition, triple-positive aPL testing, and aggressive multimodal immunosuppression with anticoagulation is life-saving. Clinicians in India should maintain a high index of suspicion for CAPS in post-COVID patients with multi-organ thrombosis.
Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson's syndrome, affects less than 1% of patients with antiphospholipid syndrome (APS) but carries a mortality rate exceeding 44% if untreated. It is defined by thrombotic microangiopathy involving ≥3 organs or tissues within one week in the presence of antiphospholipid antibodies (aPL).
Since the COVID-19 pandemic, there have been emerging reports of CAPS triggered by SARS-CoV-2 infection, presumably through molecular mimicry between viral proteins and phospholipid-binding proteins, coupled with the pro-thrombotic state induced by COVID-19 endotheliopathy. However, published data from India are extremely limited, and the clinical course in South Asian patients may differ due to genetic and haematological factors.
We report a case of CAPS in a young Indian female, precipitated by COVID-19, with simultaneous involvement of pulmonary, cerebral, renal and peripheral vascular systems — highlighting the diagnostic pitfalls and management complexities encountered in a resource-limited tertiary setting.
A 28-year-old female homemaker, with no prior medical history, presented to the emergency department of AIIMS Bhopal with a 3-day history of progressively worsening breathlessness (NYHA Class III), right calf pain and swelling, and confusion (GCS 13/15). She had tested RT-PCR positive for SARS-CoV-2 10 days prior with mild respiratory symptoms managed at home with symptomatic therapy. She was nulliparous with no previous thrombotic events. Family history was unremarkable. She was a non-smoker and denied use of oral contraceptives.
On examination: temperature 38.2°C, pulse 118/min, BP 88/60 mmHg (hypotensive), SpO₂ 82% on room air. She was tachypnoeic (RR 28/min) with bilateral coarse crepitations on auscultation. Right calf was swollen, tender and erythematous. Neurological examination revealed confusion, dysarthria and right-sided pronator drift. Fundoscopy showed bilateral papilloedema. Skin examination revealed livedo reticularis over both lower limbs.
Laboratory investigations: Hb 9.2 g/dL, WBC 14,800 cells/mm³, platelet count 54,000/mm³ (thrombocytopenia). PT/INR 1.8, aPTT 68 sec (prolonged). D-dimer >10,000 ng/mL. Serum creatinine 3.8 mg/dL (AKI). C-reactive protein 148 mg/L. Urine: 3+ proteinuria, red cell casts.
Antiphospholipid antibody panel: Lupus anticoagulant POSITIVE; anti-cardiolipin IgG 86 GPL U/mL (strongly positive); anti-β2-glycoprotein-I IgG 74 U/mL (strongly positive) — triple positivity.
Imaging: CT pulmonary angiography — bilateral segmental pulmonary emboli. Lower limb Doppler — extensive DVT right popliteal to femoral vein. MRI brain — superior sagittal sinus thrombosis with cortical venous infarction. CT abdomen — bilateral renal cortical ischaemia.
CAPS diagnosis was confirmed (definite Asherson's criteria: >3 organs, <1 week, histopathological confirmation not feasible but radiological evidence used per modified criteria). Treatment instituted: (1) Unfractionated heparin infusion (therapeutic aPTT 60–100 sec) followed by warfarin (target INR 3.0–4.0); (2) Intravenous methylprednisolone 1 g/day × 3 days; (3) Intravenous immunoglobulins 0.4 g/kg/day × 5 days; (4) Therapeutic plasma exchange — 5 sessions on alternate days; (5) Hydroxychloroquine 400 mg/day commenced after hemodynamic stabilisation.
By day 7, platelet count recovered to 1,20,000/mm³. Creatinine improved to 1.6 mg/dL. SpO₂ normalised to 97% on room air. GCS improved to 15/15 by day 10. Repeat MRI brain at 4 weeks showed near-complete resolution of sinus thrombosis. The patient was discharged on day 22 on warfarin (INR 3.2), hydroxychloroquine, and low-dose prednisolone. At 6-month follow-up, she remains well with no recurrence.
CAPS represents a distinct and devastating subtype of APS. The pathophysiology involves a cytokine storm, complement activation, and extensive thrombotic microangiopathy. COVID-19 generates a hypercoagulable milieu through endothelial damage, complement activation, and high titre pro-inflammatory cytokines — an ideal storm for CAPS precipitation.
In our patient, the presence of triple-positive antiphospholipid antibodies (lupus anticoagulant + anti-cardiolipin + anti-β2-GPI) confers the highest thrombotic risk profile. This phenotype was previously unrecognised, supporting COVID-19 as the precipitant rather than a recurrence. A prior systematic review of 500 CAPS cases (Cervera et al., Arthritis Rheum 2010) identified infections as precipitants in 22% — viral triggers including influenza, EBV, and HBV are well documented.
Treatment with the triple therapy combination (anticoagulation + corticosteroids + IVIG) achieves recovery in 63% of cases per the CAPS Registry. Addition of plasma exchange in refractory cases improves survival further. In our resource-limited setting, the decision to perform plasma exchange despite logistical constraints was critical. The use of hydroxychloroquine for secondary prevention is supported by class B evidence in aPL-positive patients.
This case highlights three critical learning points for Indian physicians: (1) Triple aPL testing should be performed in all post-COVID patients with multi-organ thrombosis; (2) CAPS must be distinguished from other thrombotic microangiopathies (TTP, HUS, DIC) by the aPL profile; (3) Early aggressive multimodal therapy is life-saving — delays in diagnosis or treatment carry high mortality.
COVID-19–precipitated CAPS, while rare, is a potentially fatal condition that can affect young, otherwise healthy individuals. This case underscores the importance of routine aPL antibody screening in post-COVID multi-organ thrombosis and demonstrates that aggressive multimodal therapy — even in resource-limited settings — can achieve excellent outcomes. CAPS should be on the differential diagnosis of every physician encountering post-COVID thrombotic emergencies.
Learning Points / Take-Away Lessons
Patient Consent
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Ethics
All procedures were in accordance with the ethical standards of the AIIMS Bhopal Institutional Ethics Committee (IEC No: AIIMS-B/IEC/2024/142) and with the 1964 Helsinki Declaration and its later amendments.
Competing Interests
None declared.
Funding
This research received no specific grant from any funding agency.
Generated with QThesis · AI-powered medical research platform · qthesis.in
© 2026 BMJ Case Reports. Open Access.
Case Report 1 of 5 · General Medicine · BMJ Case Reports