Catastrophic Antiphospholipid Syndrome Presenting Research Articles

S3428 Catastrophic Antiphospholipid Syndrome Presenting as Hepatic Injury, Pancreatitis, and Rash

S3428 Catastrophic Antiphospholipid Syndrome Presenting as Hepatic Injury, Pancreatitis, and Rash

P01 Catastrophic antiphospholipid syndrome presenting as diffuse peripheral and central thromboses

P01 Catastrophic antiphospholipid syndrome presenting as diffuse peripheral and central thromboses

Case of catastrophic antiphospholipid syndrome presenting as neuroretinitis and vaso-occlusive retinopathy

BackgroundOcular involvement in catastrophic antiphospholipid syndrome (CAPS), a rare, life-threatening form of antiphospholipid syndrome (APS) that results in multiorgan failure and a high mortality rate, has rarely been reported.Case presentationA 15-year-old girl presented with sudden vision blurring in both eyes. She had marked optic disc swelling and macular exudates in the right eye and intra-arterial white plaques, a few retinal blot hemorrhages, and a white ischemic retina in the left eye. Systemic examination revealed she had acute kidney injury with thrombotic microangiopathy (TMA), multiple cerebral infarcts, valvular dysfunction, and a high titer of triple aPL. Thus, she was diagnosed with CAPS involving the brain, eyes, heart, and kidneys. Plasma exchange and the administration of glucocorticoids, immunoglobulin, warfarin, and rituximab brought a sustained recovery of the TMA, visual symptoms, and echocardiographic findings.ConclusionsOcular involvement of both vaso-occlusive retinopathy, an APS-related thrombotic microangiopathy, and neuroretinitis, a non-thrombotic microangiopathy, can occur as an initial presentation of CAPS.

Rare Presentation of Catastrophic Antiphospholipid Syndrome with Myocarditis in Post-partum Period: Case Report and Review of Literature.

Rare Presentation of Catastrophic Antiphospholipid Syndrome with Myocarditis in Post-partum Period: Case Report and Review of Literature.

Catastrophic Anti-phospholipid Syndrome Presenting with Symmetrical Peripheral Gangrene

Catastrophic Anti-phospholipid Syndrome Presenting with Symmetrical Peripheral Gangrene

Catastrophic antiphospholipid syndrome presenting as bilateral central retinal artery occlusions.

A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial “boxcarring,” retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient's vision improved and remained stable at 20/200 and 20/80.

Catastrophic Antiphospholipid Syndrome Presenting With Complete Aortic Occlusion and Diffuse TMA in the Kidney

Catastrophic Antiphospholipid Syndrome Presenting With Complete Aortic Occlusion and Diffuse TMA in the Kidney

Catastrophic antiphospholipid syndrome presenting as venous retinal oclussion

Catastrophic antiphospholipid syndrome presenting as venous retinal oclussion

A case of catastrophic antiphospholipid syndrome presenting with acute respiratory distress syndrome as the initial manifestation

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by a combination of arterial or venous thrombosis and recurrent fetal loss, accompanied by elevated titers of antiphospholipid antibodies (aPL). Catastrophic antiphospholipid syndrome (CAPS) is a small subset of APS characterized by widespread systemic thrombotic disease with multiorgan failure. We herein describe an autopsy case of CAPS who developed severe respiratory failure due to acute respiratory distress syndrome (ARDS) as the initial manifestation. Patients with APS may exhibit a broad spectrum of pulmonary diseases. ARDS is the common pulmonary complication in CAPS, although it rarely occurs in APS. Some mechanisms of ARDS in CAPS have been postulated but the precise mechanism is still not clearly understood. It is important to understand that APS or CAPS could be a cause of ARDS since ARDS might develop as the initial manifestation of APS or CAPS as seen in our case. Our case is interesting in that severe respiratory failure due to ARDS was the initial presentation of CAPS.

Catastrophic Antiphospholipid Syndrome Presenting With Multiorgan Failure and Gangrenous Lesions of the Skin

A 38-year-old woman with a 20-year history of systemic lupus erythematosus and positive anticardiolipin antibodies developed anuria and hypotension. 20 days before, she had discontinued 25 mg prednisone, but not warfarin, on medical advice. 3 days before admission, she developed extensive necrosis and blisters involving both arms and legs and a multiorgan failure. She improved after immunosuppressive and antibiotic therapy, 3 sessions of dialysis and 5 sessions of plasmapheresis. It was decided that she could be discharged after 45 days. Her skin lesions were complicated by several abscesses requiring surgical drainage and finally healed almost completely within 9 months. Catastrophic antiphospholipid syndrome is a distinctly rare dramatic condition characterized by widespread thrombosis of small vessels, which in the present case was likely triggered by the abrupt withdrawal of steroid therapy. It should be borne in mind in cases of multiorgan failure, which does not recognize a well-defined etiology.