The relevance of “non-criteria” clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features

Abreu, Mirhelen Mendes de; Danowski, Adriana; Wahl, Denis; Amigo, Mary Carmen; Tektonidou, Maria G.; Pacheco, Marcelo S.; Fleming, Norma Regina Pereira; Domingues, Vinicius; Sciascia, Savino; Lyra, Julia O.; Petri, Michelle; Khamashta, Munther A.; Levy, Roger A.

doi:10.1016/j.autrev.2015.01.002

Cited by 200 publications

(151 citation statements)

References 75 publications

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“…At the same time, the systemic nature of APS is highlighted by other associated features include cytopenias, cognitive dysfunction, cardiac valve disease, renal impairment, and skin ulcers [2]**. Patients with APS have circulating antiphospholipid antibodies, as measured by assays for anticardiolipin IgG or IgM; anti-beta-2 glycoprotein I (β 2 GPI) IgG or IgM; or lupus anticoagulant [1]; at least one of these tests should be durably positive to classify a patient as having APS.…”

Section: Introductionmentioning

confidence: 99%

Treatment of catastrophic antiphospholipid syndrome

Kazzaz

McCune

Knight

2016

Current Opinion in Rheumatology

110

113

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Purpose of review Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of APS. While affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS. Recent findings Data from the international “CAPS registry,” spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and associated/precipitating diagnoses. Current guidelines also continue to support a role for anticoagulants and glucocorticoids as foundation therapy in all patients. Finally, new basic science and case series suggest that novel therapies, such as rituximab and eculizumab warrant further study. Summary Attention to associated diagnoses such as infection and systemic lupus erythematosus (SLE) are critical at the time of diagnosis. All patients should be treated with anticoagulation, corticosteroids, and possibly plasma exchange. In patients with SLE, cyclophosphamide should also be considered. In refractory or relapsing cases, new therapies such as rituximab and possibly eculizumab may be options, but need further study.

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Section: Introductionmentioning

confidence: 99%

Treatment of catastrophic antiphospholipid syndrome

Kazzaz

McCune

Knight

2016

Current Opinion in Rheumatology

110

113

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“…According to diagnostic criteria, the patient did not have ‘criteria’ APS, which leads to considering the possibility of ‘non-criteria’ APS. A report suggests a direct interaction between aPL and spinal cord phospholipids rather than a thrombotic process leading to ischaemia 8. As is expected, APS seems to be one of the most plausible aetiologies for myelitis, even though it is ‘non-criteria’ APS.…”

Section: Discussionmentioning

confidence: 82%

Longitudinally extensive transverse myelitis with anti-NMDA receptor antibodies during a systemic lupus erythematosus flare-up

et al. 2015

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Transverse myelitis (TM) with systemic lupus erythematosus (SLE) has been linked to the presence of autoantibodies (eg, antiaquaporin 4 (AQP4) and anticardiolipin (aCL)) and SLE-induced secondary vasculitis, but the aetiology remains incompletely understood. A 48-year-old Japanese man with a 6-year history of poorly controlled SLE had stopped glucocorticoid therapy 1 year before admission. 3 days before admission, he developed flaccid paraplegia. Spinal MRI showed a longitudinally hyperintense T2 grey matter lesion from the level of Th4 to the conus medullaris, which was considered longitudinally extensive TM (LETM). We administered steroid pulse therapy (methyl-prednisolone 1000 mg/day) for 3 days and prednisolone 50 mg/day. The patient's flaccid paralysis gradually improved. We concluded that the patient's TM was caused by SLE flare-up, even though we could not completely rule out antiphospholipid syndrome. SLE myelitis is relatively rare and many aetiologies are possible for TM in SLE.

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“…Under APS, we also included patients with high-titre antiphospholipid antibody and the following non-criteria manifestations: echocardiographically or MRI-documented cardiac valve disease, typical MRI-documented brain disease not otherwise explained, histopathologically documented APS nephropathy or leg ulcers not otherwise explained 10. In the tables, patients with two or more of SLE, RA, Sjögren's syndrome or APS diagnoses are included in each relevant diagnosis.…”

Section: Methodsmentioning

confidence: 99%

Patients with overlap autoimmune disease differ from those with ‘pure’ disease

2015

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ObjectiveTo determine frequency, demographic and treatment characteristics of patients with an overlapping second autoimmune illness (2nd AI).MethodsWe analysed two cohorts containing 897 patients with ‘pure’ systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Sjögren's syndrome or antiphospholipid syndrome (APS) and 424 patients with one of these diagnoses plus at least one 2nd AI.ResultsA 2nd AI occurred in 38% of all patients diagnosed as having SLE (with or without a 2nd AI), 30% with RA, 52% with Sjögren's syndrome and 43% with APS. Compared to those without 2nd AI, patients with 2nd AI differ in age, sex, race and treatment at last visit.IntepretationThese differences may have important implications for understanding treatments, outcomes and mechanisms of SLE and related diseases.

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The relevance of “non-criteria” clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features

Cited by 200 publications

References 75 publications

Treatment of catastrophic antiphospholipid syndrome

Treatment of catastrophic antiphospholipid syndrome

Longitudinally extensive transverse myelitis with anti-NMDA receptor antibodies during a systemic lupus erythematosus flare-up

Patients with overlap autoimmune disease differ from those with ‘pure’ disease

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