Initial Manifestation Of Antiphospholipid Syndrome Research Articles

Chorea as the Initial Manifestation of Antiphospholipid Syndrome (P9-5.020)

<h3>Objective:</h3> To discuss a rare case of chorea presenting as the initial manifestation of autoimmune disease. <h3>Background:</h3> Autoimmune chorea, including paraneoplastic, post-infectious, and idiopathic etiologies, is one of the leading causes of adult-onset chorea behind Huntington’s and vascular diseases.¹ In particular, in primary antiphospholipid syndrome (APS) and systemic lupus erythematosus, chorea is the initial manifestation of disease in 0.9 to 2% of cases.^1,2 <h3>Design/Methods:</h3> Verbal and written consent were obtained from the patient. <h3>Results:</h3> 73-year-old female with PMH of ITP, left MCA ischemic stroke, SDH, and DVT on anticoagulation who presented initially to ED with choreoathetoid movements starting 3 days prior to admission. History was unremarkable for triggering factor. Head imaging was unremarkable for acute intracranial process. B12, folate, TSH, electrolytes, RPR, serum copper, ANA, anti-dsDNA, SS-A, SS-B, Smith, and RNP antibodies negative. Lupus anticoagulant, β2 glycoprotein, and anti-cardiolipin antibodies positive. EEG unremarkable. Pelvic ultrasound negative. Genetic testing for Huntington’s negative. LP normal with exception of positive Coxsackie B1–B6 and A24 antibodies. After five-day course of IVIg, movements improved and she was discharged on no immunosuppression. Five weeks later, patient returned to ED with worsening of choreoathetoid movements. Repeat head CT again negative for acute pathology. Repeated spinal was unremarkable. Patient received Solumedrol 1g for four days with significant improvement in symptoms, and discharged on Prednisone 60 mg daily with recommendation to follow-up with rheumatology and neurology. On follow-up several months later, patient maintained on Prednisone 5 mg daily with no recurrence of choreoathetoid movements. <h3>Conclusions:</h3> While chorea is not the most commonly reported movement disorder in the context of autoimmune disease, it is often a delayed diagnosis, resulting in recurrent admissions and healthcare costs. Laboratory testing for autoimmune disorders should be included in the initial work-up of new-onset chorea to establish the diagnosis and properly treat as to prevent development of fulminant disease. <b>Disclosure:</b> Ms. Alayon has nothing to disclose. Mrs. Goodis has nothing to disclose. Dr. Feinberg has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acadia. Dr. Feinberg has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Catalyst. Dr. Feinberg has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for eisai. Dr. Feinberg has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Argenx. Dr. Feinberg has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Abbvie. Dr. Feinberg has stock in Amarin. Stuart N. Kieran, MD has nothing to disclose. Dr. Wirkowski has nothing to disclose.

Letter by Cardenas Suri and Jimomila Bening Regarding Article, “Acute Heart Failure in Multisystem Inflammatory Syndrome in Children in the Context of Global SARS-CoV-2 Pandemic”

HomeCirculationVol. 143, No. 10Letter by Cardenas Suri and Jimomila Bening Regarding Article, “Acute Heart Failure in Multisystem Inflammatory Syndrome in Children in the Context of Global SARS-CoV-2 Pandemic” Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBLetter by Cardenas Suri and Jimomila Bening Regarding Article, “Acute Heart Failure in Multisystem Inflammatory Syndrome in Children in the Context of Global SARS-CoV-2 Pandemic” Hisyovi Cardenas Suri and David Jimomila Bening Hisyovi Cardenas SuriHisyovi Cardenas Suri https://orcid.org/0000-0002-0933-8716 Tamale Teaching Hospital, Ghana. Search for more papers by this author and David Jimomila BeningDavid Jimomila Bening https://orcid.org/0000-0002-5877-4741 Tamale Teaching Hospital, Ghana. Search for more papers by this author Originally published8 Mar 2021https://doi.org/10.1161/CIRCULATIONAHA.120.049139Circulation. 2021;143:e757–e758To the Editor:We recently read the article in Circulation by Belhadjer et al1 about the multisystem inflammatory syndrome and the relation with cardiac events. This problem goes far beyond the cardiac problem to include other systemic conditions.The presence of several reports of hyperinflamatory syndrome in children, even recognized and with diagnostic criteria according to a report by the World Health Organization and Centers for Disease Control and Prevention like multisystem inflammatory syndrome in children and adolescents,2 has led us to review various diseases that present with phenomena of organ failure, thrombosis, cytokine storm, and elevation of ferritin, mainly involving the lung, heart, kidney, skin, and so forth. Among the possible causes, we find a common entity, the catastrophic antiphospholipid antibody syndrome.In the CAPS Registry (Catastrophic Antiphospholipid Antibody Syndrome), catastrophic antiphospholipid antibody syndrome as the initial manifestation of antiphospholipid syndrome occurred in 86.6% of children, with infections being the triggering factor in 60.9% of the cases. Cardiac involvement was present in 57.4% of children and included cardiac failure, heart valve lesions, lung (63.0%), skin (37.0%), and gastrointestinal (17.4%).3Infections in children are more frequent than in adults, which have been shown to play a role in the theory of “molecular mimicry” (molecular imitation), where certain viruses and bacteria that have an amino acid sequence similar to that of B2-GP1 result in an immune response producing antiphospholipid antibodies. B2-GP1 could activate the immune response through interaction with a membrane receptor (toll-like receptors), and from this a series of signals are generated that increase the production of proinflammatory cytokines.4The medical community recognizes the multisystem inflammatory syndrome, but most do not agree with the diagnosis of Kawasaki disease. In addition, the patients do not meet the diagnostic criteria to support macrophage activation syndrome; however, clinically the patients meet the catastrophic antiphospholipid antibody syndrome criteria,4 and it would only be necessary to indicate antinuclear antibodies to confirm this diagnosis.Disclosures None.Footnoteshttps://www.ahajournals.org/journal/circ

Bilateral Adrenal Hemorrhage and Coagulation Laboratory Abnormalities: A Case Report of a Man with Enigmatic Clinical Features.

Background: bilateral adrenal hemorrhage can cause adrenal insufficiency. Antiphospholipid syndrome (APS) is considered a rare etiology of bilateral adrenal hemorrhage with a relatively small number of case reports. APS can be secondary to other autoimmune diseases such as SLE in which cardiac manifestations are common.&#x0D; Case presentation: a fifty-four-year-old male presents with left flank pain as a result of unilateral adrenal hemorrhage, a few days later, bilateral hemorrhage is documented in the presence of abnormal coagulation state and other laboratory clues. APS diagnosis was established.&#x0D; Coexisting chest pain accompanied by diffused ST elevation, long QT, slightly high troponin with a high pro-BNP, in addition to echo’s findings such abnormal regional wall abnormality and the absence of pericardial effusion made the case more interesting, differential diagnosis was proposed in accordance.&#x0D; Conclusion: bilateral hemorrhage in APS considered rare. Requiring a high index of clinical suspicion. Particularly when it is the initial manifestation of APS as in our case. Underlying autoimmune diseases should be screened actively, especially when other unexplained manifestations are presented.

Autonomic neuropathy-in its many guises-as the initial manifestation of the antiphospholipid syndrome.

Autonomic disorders have previously been described in association with the antiphospholipid syndrome. The present study aimed to determine the clinical phenotype of patients in whom autonomic dysfunction was the initial manifestation of the antiphospholipid syndrome and to evaluate for autonomic neuropathy in these patients. This was a retrospective study of 22 patients evaluated at the University of Colorado who were found to have a disorder of the autonomic nervous system as the initial manifestation of antiphospholipid syndrome. All patients had persistent antiphospholipid antibody positivity and all patients who underwent skin biopsy were found to have reduced sweat gland nerve fiber density suggestive of an autonomic neuropathy. All patients underwent an extensive evaluation to rule out other causes for their autonomic dysfunction. Patients presented with multiple different autonomic disorders, including postural tachycardia syndrome, gastrointestinal dysmotility, and complex regional pain syndrome. Despite most having low-titer IgM antiphospholipid antibodies, 13 of the 22 patients (59%) suffered one or more thrombotic event, but pregnancy morbidity was minimal. Prothrombin-associated antibodies were helpful in confirming the diagnosis of antiphospholipid syndrome. We conclude that autonomic neuropathy may occur in association with antiphospholipid antibodies and may be the initial manifestation of the syndrome. Increased awareness of this association is important, because it is associated with a significant thrombotic risk and a high degree of disability. In addition, anecdotal experience has suggested that antithrombotic therapy and intravenous immunoglobulin therapy may result in significant clinical improvement in these patients.

PS100 Heart Failure as an Initial Manifestation of Antiphospholipid Syndrome

PS100 Heart Failure as an Initial Manifestation of Antiphospholipid Syndrome

Splenic Infarction as the Initial Manifestation of Antiphospholipid Syndrome in a Systemic Lupus Erythematosus Patient

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. In patients with SLE, the prevalence of antiphospholipid antibodies is considerably higher, and is largely responsible for thrombosis. Splenic infarction is a rare complication of arterial thrombosis in patients with SLE. It is important to consider splenic infarction in a patient with SLE complaining of left upper quadrant (LUQ) pain because of the possibility of severe infarction-related complications, such as subcapsular hemorrhage and splenic rupture. We report a case of solitary splenic infarction in a patient with SLE. The only symptom was LUQ pain of 3-day duration. Lupus anticoagulant activity was positive and abdominal-pelvic computed tomography (CT) was consistent with splenic infarction. She did not show any other evidence of thrombotic events. The patient was diagnosed with antiphospholipid syndrome that presented as a splenic infarction in a SLE patient. (Korean J Med 2014;86:651-654)

English

AIM: To report a case of Central Retinal Artery Occlusion (CRAO) in young males in its initial manifestation as Primary Antiphospholipid Syndrome. METHODS: 32 year healthy male, with abrupt sudden painless loss of vision in right eye since 48 hours, with Grade 2 Relative afferent pupillary defect, visual acuity of hand movements in OD and 6/18 in OS. Fundoscopy disclosed signs compatible of central retinal artery occlusion confirmed with FFA. Carotid Doppler imaging and echocardiography was done to determine the source. RESULTS: Antiphospholipid antibody cofactor, beta2-glycoprotein 1 antibodies, IgM, was positive with titre of more than 94 un its/ml on two occasions, 12 weeks apart, with normal range being less than 20 units/ml for each isotope (IgG, IgM, or IgA).According to the 2006 revised Sapporo criteria Antiphospholipid syndrome was diagnosed. Thorough examination excluded other system involvement. Immunological studies excluded other systemic disorders. CONCLUSIONS: In literature, prevalence of CRAO is 0.85% for every 100000 and prevalence of Antiphospholipid Syndrome in patients showing a major retinal vascular obstruction is 5% - 33%. Antiphospholipid syndrome should be ruled out in every young patient who presents with Central retinal artery occlusion. Association must be considered, as Central retinal artery occlusion could be the initial manifestation of antiphospholipid syndrome with high risk of recurrence.

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.

A Case of Primary Antiphospholipid Syndrome with Pulmonary Thromboembolism

Antiphospholipid syndrome (APS) causes recurrent thromboses and morbidity during pregnancy, including fetal loss. This malady is associated with the persistent presence of anticardiolipin antibody or lupus anticoagulant. The pulmonary manifestations of antiphospholipid syndrome include pulmonary thromboembolism, pulmonary hypertension, acute respiratory distress syndrome, etc. Pulmonary thromboembolism is often the initial manifestation of antiphospholipid syndrome and a timely diagnosis is critical due to the high mortality rate. We herein report on a 19-year-old man with pulmonary thromboembolism that was caused by primary antiphospholipid syndrome. He presented with blood-tinged sputum, fever and epigastric pain, and his chest computerized tomography showed pulmonary thromboembolism. The other possible causes of pulmonary thromboembolism were excluded and the diagnosis of primary antiphospholipid syndrome was confirmed by the lupus anticoagulant that was present on two occasions six weeks apart. We also discuss the nature and management of antiphospholipid syndrome, along with a brief review of the relevant literatures.