Pontine Infarct as Initial Presentation of Catastrophic Antiphospholipid Syndrome in Systemic Lupus Erythematous

Rathika Rajah, Rizna A Cader


Antiphospholipid syndrome (APLS) is an autoimmune condition which commonly manifests as an arterial or venous thrombosis affecting medium to large vessels, with the presence of antiphospholipid antibodies. APLS can be a primary disease by itself, or secondary to other autoimmune diseases, such as Systemic Lupus Erythematosus (SLE). Catastrophic APLS is a rare but a fatal sequelae of APLS, affecting up to three or more organs, and progresses rapidly with a high mortality rate. We report a case of catastrophic APLS in a young woman with underlying SLE who presented to us with multiple cranial nerve palsies due to bilateral pontine infarct, and eventually developed deep vein thrombosis and pulmonary embolism during the course of the illness. She was treated with high dose corticosteroids and intravenous cyclophosphamide with biochemical improvement. In this case report, we would like to highlight the fact that our patient had bilateral pontine infarcts as the initial presentation, with no inciting events and  antiphospholipid antibodies were negative during the acute illness.


Antiphospholipid Syndrome; Catastrophic APLS; Infarct; Thrombosis; Cyclophosphamide


Koike T. Antiphospholipid syndrome: 30 years and our contribution. Int J Rheum Dis. 2015;18(2):233-41.

Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol. 2014;3(1):9.

Bucciarelli S, Espinosa G, Cervera R, Erkan D, Gómez‐Puerta JA, Ramos‐Casals M, Font J, Asherson RA. Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis & Rheumatism. 2006;54(8):2568-76.

Asherson RA. Multiorgan failure and antiphospholipid antibodies: the catastrophic antiphospholipid (Asherson’s) syndrome. Immunobiology. 2005;210(10):727-33.

Asherson RA, Cervera R, De Groot PG, Erkan D, Boffa MC, Piette JC, Khamashta MA, Shoenfeld Y. Catastrophic Antiphospholipid Syndrome Registry Project Group. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530-4.

Aguiar CL, Erkan D. Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease. Therapeutic advances in musculoskeletal disease. 2013;5(6):305-14.

Ortel TL, Kitchens CS, Erkan D, et al. Clinical causes and treatment of the thrombotic storm. Expert Review of Hematology. 2012;5(6):653-9.

Fleetwood T, Cantello R, Comi C. Antiphospholipid syndrome and the neurologist: from pathogenesis to therapy. Frontiers Neurol. 2018;9.

Mull ES, Aranez V, Pierce D, Rothman I, Abdul-Aziz R. Newly diagnosed systemic lupus erythematosus: atypical presentation with focal seizures and long-standing lymphadenopathy. JCR: Journal of Clinical Rheumatology. 2019;25(7):e109-13.

Miret C, Cervera R, Reverter JC, et al. Antiphospholipid syndrome without antiphospholipid antibodies at the time of the thrombotic event: transient’seronegative’antiphospholipid syndrome?. Clinical and Experimental Rheumatology. 1997;15(5):541-4.

Drenkard C, Sanchez-Guerrero J, Alarcon-Segovia D. Fall in antiphospholipid antibody at time of thromboocclusive episodes in systemic lupus erythematosus. Journal Rheumatol. 1989;16(5):614-7.

Cervera R, Rodríguez-Pintó I, Colafrancesco S, et al. 14th international congress on antiphospholipid antibodies task force report on catastrophic antiphospholipid syndrome. Autoimmunity Rev. 2014;13(7):699-707.

Elagib EM, Eltahir NI, Adam ME, Mahmoud ZI, Yousif HH. Catastrophic antiphospholipid syndrome in combination with SLE treated by Rituximab: A case report and literature review. Lupus: Open Access. 2019;4:137.

Bucciarelli S, Erkan D, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse. Clinical Reviews Allergy & Immunology. 2009;36(2-3):80-4.

Full Text: PDF


  • There are currently no refbacks.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.