History Of Systemic Lupus Erythematosus Research Articles

Ankle ulcer as a complication of systemic lupus erythematosus: A case report

Background: In 10-20% of cases, Raynaud’s Phenomenon (RP) is an initial manifestation of underlying connective tissue disease such as systemic lupus erythematosus (SLE), then Raynaud’s Phenomenon on SLE called secondary RP. Case presentation: A 26-year-old female came with pain in the fingers and toes. Pain aggravated by activity and cold temperatures. there are black-coloured changes at the tips of the toes, pale and bluish at bilateral fingertips. History of systemic lupus erythematosus since last 3 months. Physical examination revealed bilateral pale and cyanotic fingers, Black-colored sores on the right lateral malleolus, 1st and 5th toe of the right foot, 2nd and 3rd toes of the left foot. There is tenderness and pitting oedema. Peripheral pulses are not palpable. The antinuclear Antibody (ANA) profile is positive. Multislice computed tomography (MSCT) Angiography of the bilateral lower extremity was performed where there is stenosis proximal right dorsalis pedis artery, stenosis right plantar pedis artery, stenosis 1/3 medial left tibial posterior artery, and stenosis 1/3 distal left tibial anterior artery. The patient was diagnosed with secondary RP because of SLE and we performed debridement of the wound, then treated the patient with calcium channel blocker, prostacyclin analogue, antiplatelet and non-pharmacological treatment included avoiding cold exposure. Symptom resolution and ANA titer reduction are obtained at intervals of two- and six-months post-treatment. Conclusion: RP in this case is a manifestation of systemic lupus erythematous. Ulcer in the lateral malleolus area is a complication of secondary RP.

P1702 Libman-Sacks endocarditis of the mitral valve combined with right atrial thrombus formation

Abstract Case presentation A 46-years old female with a history of systemic lupus erythematosus (SLE) was admitted to hospital care after the manifestation of a tonic-clonic generalized seizure. Since this had been the first neurological event, a thorough diagnostic work-up was performed. CT- and MRI-imaging of the brain revealed cerebral microangiopathy and two small fresh ischemic lesions in the left frontal and temporobasal regions, respectively. While the microangiopathy could be reconciled with cerebral SLE-vasculitis, the ischemic lesions pointed to thromboembolism whose source could be potentially cardiogenic. Findings. In fact, the transesophageal echocardiogram showed a small vegetation (5x8 mm) on the posteromedial cusp of the posterior mitral leaflet (P3) with moderate regurgitation, likely to represent Libman-Sacks endocarditis and a potential source of systemic embolization. In addition, a mass of was visible protruding from the fossa ovalis into the right atrium, see figure. It represented a thrombus according to MRI which was subsequently performed. Since no interatrial passage of microbubbles occurred, the foramen ovale was unlikely to be patent and to allow for paradoxical embolism into the brain. However, the right atrial thrombus was compatible with a history of repeated deep venous thrombosis and pulmonary embolism in the recent past. Discussion This case exemplifies the combination of Libman-Sacks endocarditis on the mitral valve with right atrial thrombus formation and ensuing embolism both into the venous and arterial system. It demonstrates the importance of closely monitoring and treating coagulopathies in SLE patients which makes them prone to thrombus formation both in the systemic and pulmonary circulation. Abstract P1702 Figure.

Rare Case of Bilateral Patellar Tendon Rupture in a Patient of Systemic Lupus Erythematosus with Chronic Renal Failure

Unilateral patellar tendon ruptures are a common complication that can occur frequently among athletic patients below the age of 40; however, bilateral spontaneous patellar tendon ruptures are rare and still not fully understood. We present a case of bilateral patellar tendon rupture in a 37-year-old patient with a history of systemic lupus erythematosus (SLE) who had concomitant chronic renal failure. Primary repair utilizing the Krackow interlocking stitch technique provided excellent outcomes in both knees. This case highlights the rare occurrence of spontaneous bilateral patellar tendon rupture in patients with SLE and chronic renal failure.

Pseudo-Pseudo Meigs’ Syndrome in a Patient with Systemic Lupus Erythematosus Flare

Pseudo-pseudo Meig’s syndrome (PPMS) is an exceedingly uncommon disease in patients with diagnosed systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD). This is a case report of a 23-year-old woman with a history of systemic lupus erythematosus (SLE), presented with rapidly increasing abdominal girth, facial/pedal edema, oliguria, and dyspnea, who was presumed to be developing pre-eclampsia in her 35th week of gestation. She was managed by an emergency Cesarean-section, and an incidental finding of peri-operative ascitic fluid was obtained and a right-sided pleural effusion. Upon further investigations, her CA-125 levels were found to be elevated. Her anti-double-stranded DNA titers were increased whereas complement levels (C3 and C4) were reduced indicating SLE flare. All these features suggested the diagnosis of pseudo-pseudo meig’s syndrome, a rare condition associated with SLE. It is asserted in our case that her pregnancy triggered the lupus flare.

Annals for Educators - 19 November 2019.

Annals for Educators - 19 November 2019.

"SAGE-IN-BUD": A RARE CAUSE OF TREE-IN-BUD PULMONARY INFILTRATES

SESSION TITLE: Case Report Semifinalists 3 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Tree-in-bud infiltrates on lung imaging may be caused by a number of infectious, neoplastic, congenital, and inflammatory etiologies, however is very rarely secondary to foreign body aspiration. We present a case with an unusual cause of diffuse pulmonary tree-in-bud infiltrates. CASE PRESENTATION: A 55-year-old female with a past medical history of systemic lupus erythematosus, diffuse alveolar hemorrhage, and lupus anticoagulant syndrome presented to the emergency department with a three-day history of a dry cough. A computed tomography (CT) scan of her chest revealed diffuse, uniform tree-in-bud infiltrates, involving every lobe of her lungs. The patient had a chronic, intermittent mild, dry cough, but an otherwise unremarkable review of systems. She was a never smoker and denied any other inhalation or illicit drug use. Her previous episode of diffuse alveolar hemorrhage occurred 13 months prior to this admission and had not recurred. Laboratory evaluation including complete blood count, metabolic profile, and HIV testing was unremarkable. Her vitals were normal, as were pulmonary function tests. .Bronchoscopy revealed a macrophage-predominant bronchoalveolar lavage sample, negative cultures, no acid fast bacteria, and cytology that was negative for malignancy. Transbronchial biopsies revealed extensive small airway impaction by foreign material with associated foreign-body giant cell reaction and granulomatous inflammation. Further evaluation demonstrated a positive reaction on a Periodic Acid-Schiff modification (PAS) of the foreign material. Given the histological appearance of the foreign body and positive PAS staining, forensic pathology determined that the foreign material seen on biopsy was consistent with plant material. An extensive exposure history was obtained and it was revealed that, as a part of her Native American religion, she would semi-regularly burn a bundle of white sage (Salvia apiana) leaves. While burning the bundle of white sage she would walk around her home, spreading the smoke in a ritual termed “smudging,” admittedly inhaling the smoke and particulate matter from the burning sage. DISCUSSION: Given her inhalation exposure to particulate plant matter, a paucity of other exposures, and the findings on transbronchial biopsy we believe her tree-in-bud infiltrate was secondary to her practice of “smudging.” CONCLUSIONS: To our knowledge, this is the only reported case of inhaled plant material causing an inflammatory small airway disease and a tree-in-bud infiltrates on chest imaging. Reference #1: Causes and Imaging Patterns of Tree-in-Bud Opacities. Miller, Wallace T. et al. CHEST , Volume 144 , Issue 6 , 1883 - 1892 DISCLOSURES: No relevant relationships by Vikramjit Khangoora, source=Web Response Speaker/Speaker's Bureau relationship with Genentech Please note: $5001 - $20000 Added 03/15/2019 by Joshua Sill, source=Web Response, value=Honoraria

Infección por Listeria monocytogenes en una mujer gestante con lupus eritematoso sistémico y síndrome HELLP

La listeriosis es una enfermedad transmitida principalmente por alimentos contaminados con Listeria monocytogenes. Se presenta con mayor frecuencia en neonatos, mayores de 65 años, mujeres gestantes y pacientes inmunosuprimidos. La infección por L. monocytogenes durante la gestación se asocia a una importante morbimortalidad materno-fetal.
 Se reporta el caso de una mujer gestante de 29 años de edad con lupus eritematoso sistémico, a quien se le diagnosticó bacteriemia por L. monocytogenes. Durante la hospitalización, el cuadro clínico se complicó con hipertransaminasemia y, ante la presencia de trombocitopenia, se estableció el diagnóstico presuntivo de síndrome HELLP. El alto riesgo de morbimortalidad llevó a una finalización precoz dela gestación.
 La importancia de este trabajo clínico radica en presentar la dificultad en el diagnóstico y manejo en una paciente gestante de gran complejidad con una infección relativamente frecuente que puede pasar desapercibida.

Unusual cause of lower gastrointestinal bleed and abdominal pain

A young patient with a history of systemic lupus erythematosus (SLE) complicated by lupus nephritis presented to the emergency department with vomiting, diarrhoea and diffuse abdominal pain for 7 days....

Neuropsychiatric symptoms in a patient with Dyke\u2013Davidoff\u2013Masson syndrome and systemic lupus erythematosus: a case report

BackgroundDyke–Davidoff–Masson syndrome is an uncommon constellation of radiological and clinical findings. Few reports describe co-occurring psychiatric manifestations. Systemic lupus erythematosus is a systemic disease with vascular, neurologic, and psychiatric involvement. To the best of our knowledge, no case reports have been made associating these entities.Case presentationWe present the case of a 21-year-old Mexican mestizo woman with a history of systemic lupus erythematosus diagnosed at 4 years of age, who developed focal impaired awareness seizures when she was 8-years old, which became treatment-resistant at age 15. Two years prior to our evaluation, she developed deep vein thrombosis; clinical and laboratory criteria were met to diagnose secondary antiphospholipid syndrome. After being treated with anticonvulsants, glucocorticoids, and immunosuppressants with only a partial response, she developed a severe major depressive episode 1 year prior to our assessment, including two suicide attempts. She was referred to the out-patient clinic of our department for evaluation; intellectual disability, depressive symptoms, and behavioral symptoms were documented. Imaging studies revealed structural abnormalities in the left cerebral hemisphere: cortical atrophy, enlargement of sulci and cisternal spaces, and hyperpneumatization of the frontal sinus. Treatment with an antidepressant was initiated and maintained for 1 year, added to anticonvulsants and immunosuppressants. Depressive and behavioral symptoms diminished and no suicidal ideation has been noted at follow-up.ConclusionsDyke–Davidoff–Masson syndrome was diagnosed, accompanied by clinical symptoms previously reported as epilepsy and intellectual disability. This case report illustrates the complexity of syndrome presentation in an adult female, constituting a diagnostic and therapeutic challenge. This constellation of symptoms and structural brain abnormalities should be kept in mind in patients with neuropsychiatric manifestations and systemic diseases with central nervous system involvement, especially when diagnosed at a young age.

Screening characteristics for enrichment of individuals at higher risk for transitioning to classified SLE.

Further prospective study is needed to elucidate the etiology and natural history of systemic lupus erythematosus development. The clinical complexity of this heterogeneous disease makes study design challenging. Our objective was to ascertain useful screening factors for identifying at-risk individuals for follow-up rheumatologic assessment or inclusion in prospective studies. We attempted to re-contact 3823 subjects with a family history of systemic lupus erythematosus, who did not meet American College of Rheumatology systemic lupus erythematosus classification at a baseline study visit; 436 agreed to follow-up participation an average of 6.3 years after baseline. In total, 56 of these individuals had transitioned to classified systemic lupus erythematosus (≥ 4 cumulative American College of Rheumatology criteria, verified by medical record review) by the time of follow up. Generalized estimating equations assessed associations between our dichotomous outcome of transitioning to systemic lupus erythematosus with baseline characteristics, including ANA positivity, Connective Tissue Disease Screening questionnaire systemic lupus erythematosus score, and number of American College of Rheumatology criteria. We analyzed predictive accuracy of characteristics on transitioning. ANA positivity, Connective Tissue Disease Screening questionnaire systemic lupus erythematosus score categorization of possible or probable systemic lupus erythematosus, and greater number of American College of Rheumatology criteria at baseline were each associated with transitioning to systemic lupus erythematosus classification. Being ANA positive and having confirmed immunologic criteria at baseline had the highest positive predictive value and specificity for transitioning to systemic lupus erythematosus. American College of Rheumatology Connective Tissue Disease Screening questionnaire systemic lupus erythematosus score categorization of possible or probable systemic lupus erythematosus had a better positive predictive value, negative predictive value, sensitivity, and specificity than ANA positivity. Given limited resources, identifying individuals for follow up based on the systemic lupus erythematosus portion of the Connective Tissue Disease Screening questionnaire could be an efficient way to identify family members at highest risk of disease transition.

HYDROXYCHLOROQUINE CARDIOMYOPATHY IN A PATIENT WITH GENE POSITIVE HYPERTROPHIC CARDIOMYOPATHY

Hydroxychloroquine (HCQ) is a commonly used drug that has been linked to drug-induced cardiomyopathy. A 57 year old female with a history of systemic lupus erythematosus (stable on HCQ for 30 years) and known MYBPC3 gene positive hypertrophic cardiomyopathy (HCM) presented with decompensated heart

Annals for Educators - 19 February 2019.

Annals for Educators - 19 February 2019.

Acute Liver Failure and Thyrotoxicosis Managed with Liver Transplant and Thyroidectomy

Acute liver failure and hyperthyroidism are not typically common, although some cases have been reported. The mechanisms involved and optimal management are not well-defined. This article presents the case of a 32-year-old African American female referred for evaluation of abdominal pain and jaundice, with a past medical history of systemic lupus erythematosus and Grave’s disease. She had thyrotoxicosis after administration of contrast and developed acute liver failure culminating in liver transplant and subsequent total thyroidectomy with a favorable outcome.

Aspergiloma pulmonar en paciente con síndrome de sobreposición: Reporte de caso

Introducción: La infección por Aspergillus spp. representa un reto diagnóstico y terapéutico para el clínico, puesto que existe un número aproximado de 180 especies. El pulmón es el principal órgano afectado por el Aspergillus debido a la alta capacidad esporulativa de este hongo, y a que sus conidias son lo suficientemente pequeñas para poder alcanzar el alveolo. Caso: Mujer de 41 años de edad con antecedente de lupus eritematoso sistémico, ingresó con un cuadro de disfagia, en el cual se confirmó la presencia de síndrome de sobreposición mediante perfil inmunológico. Posteriormente, mediante estudio imagenológico, realizado por tener tos y disnea, se encontró la presencia de una masa ovoidea en hemitorax derecho y al realizar una fibrobroncoscopia se evidenció que dicha lesión era compatible con un aspergiloma, por lo que se dio manejo antifúngico con una evolución clínica adecuada. Conclusión: El aspergiloma se presenta como una masa voluminosa constituida por filamentos miceliales, la cual se puede confundir con múltiples patologías principalmente de tipo tumoral, por lo que es necesario realizar biopsia de la misma.

Sekundarni fosfolipidni sindrom otkriven kod pacijenta sa pneumonijom - prikaz slučaja

Introduction: Secondary antiphospholipid syndrome (SAPS) is APS that occurs in the context of another medical condition. Although antiphospholipid antibodies (aPL) can occur in patients with viral, bacterial, or protozoal infections, they are rarely associated with thrombosis. Aim: To present an uncommon case of pneumonia and pleural effusion complicated with pulmonary thromboembolism in a patient with systemic lupus erythematosus (SLE), due to non-diagnosed secondary antiphospholipid syndrome. Case report: A 28 years old woman, never pregnant, with a family history of systemic lupus erythematosus, has been suffering from SLE from the age of 18. She had only articular and skin manifestations, without internal organ involvement. She has been taking Methotrexate (10 mg weekly) and Prednisone (5 mg daily) in the previous 10 years. She was presented at the Emergency department with the radiological finding of pleuropneumonia followed by temperature up to 39C, shortness of breath, cough, fatigue, and weakness. Results: She was treated initially with three antibiotics and thoracocentesis. She developed pulmonary thrombosis without deep venous thrombosis. Laboratory findings were: ESR = 37 mm/h, CRP = 2+, ANA = 1/80 peripheral, RF negative, anti-dsDNA = 147 (positive > 40), anti-Sm negative, anticardiolipin IgG antibody 158 GPL (up to 12), anticardiolipin IgM antibody 5.5 MPL (up to 10), anti-beta2-glycoprotein IgG and IgM were negative and lupus anti-coagulant was 88" (18"-55"). The patient underwent Heparin and Warfarin treatment, by checking INR to be in a 2.6 to 3.5 range. Prednisolone and Hydroxychloroquine were also started. Conclusion: All patients suspected with SLE should be evaluated for antiphospholipid antibodies (APL). However, APS diagnosis requires both clinical and laboratory features.

Liver PEComa. An extremely rare case report with complicated post-operative course

Background: Perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal tumor with heterogeneous clinical behavior. Liver PEComas are very rare. Methods: The case of a 47-year-old man with hepatic PEComa and a complicated postoperative course is reported. Results: The patient had a history of systemic lupus erythematosus (SLE) and developed epigastric pain. An abdominal CT showed a large (7 cm) heterogeneous mass on segments I/IV, in direct contact with the inferior vena cava and portal vein. Percutaneous liver biopsy showed PEComa and the patient underwent left hepatectomy. On post-op day 7, he became pyrexial, with significant elevation in transaminases and inflammatory markers. Imaging (CT/MRI) showed a hypodense area in segment VIII close to resection margin. Blood cultures were taken and broad spectrum iv antibiotics were administered. As he continue to be pyrexial with a clinical picture of sepsis he underwent a second laparotomy, where necrosis of segment VIII was found and it was resected. As he became again pyrexial (on day 7), a new CT abdo was done revealing concentric hepatic artery stenosis with hypodense areas on segment VI with sphenoid shape. Acute exacerbation of SLE with right hepatic artery partial thrombosis or vasculitis was considered to be the diagnosis. He was commenced on steroids and anticoagulants with good clinical response. Conclusion: Management of hepatic PEComas can be very challenging especially in patients with significant co-morbidities.

Difficult-to-diagnose diabetes in a patient treated with cyclophosphamide \u2013 the contradictory roles of immunosuppressant agents: a case report

BackgroundCyclophosphamide may induce autoimmune diabetes through a decrease in suppressor T cells and increase of proinflammatory T helper type 1 response in animal models. In humans, this association is not as clear due to the presence of other risk factors for hyperglycemia, but it could be a precipitant for acute complications.Case presentationA 31-year-old Mestizo-Mexican woman with a history of systemic lupus erythematosus presented with severe diabetic ketoacidosis, shortly after initiating a multi-drug immunosuppressive therapy. She did not meet the diagnostic criteria for type 1 or type 2 diabetes and had no family history of hyperglycemic states. She persisted with hyperglycemia and high insulin requirements until the discontinuation of cyclophosphamide. After this episode, she recovered her endogenous insulin production and the antidiabetic agents were successfully withdrawn. After 1 year of follow up she is still normoglycemic.ConclusionCyclophosphamide may be an additional risk factor for acute hyperglycemic crisis. Glucose monitoring could be recommended during and after this treatment.

Trimethoprim-sulfamethoxazole induced circulatory shock in a human immunodeficiency virus uninfected patient: a case report and review

BackgroundSevere systemic reactions resembling septic shock have been described following trimethoprim-sulfamethoxazole (TMP-SMX) administration. Nearly all cases described in the literature occurred in HIV-infected patients.Case presentationWe present a 42-year-old woman with a history of systemic lupus erythematosus (SLE) who was admitted to the Intensive Care Unit (ICU) twice with fever and circulatory shock after taking a dose of TMP-SMX 800–160 mg. She had no respiratory distress, urticarial rash or eosinophilia on presentation. Infectious workup during both admissions was negative and treatment with antibiotics, steroids and vasopressors was de-escalated with clinical improvement. She was found to be HIV negative, however, labs revealed a low CD4+ count.ConclusionsTMP-SMX can rarely result in a severe, non-anaphylactic circulatory shock; if initially unrecognized, patients may undergo repeat drug exposure with an associated high morbidity risk. While more commonly reported in HIV individuals, this case demonstrates that TMP-SMX related circulatory shock can occur in a HIV negative patient.

Immunoglobulin-induced aseptic meningitis: a case report

BackgroundImmunoglobulin associated meningitis is a rare disease that mimics infectious meningitis. This is, to our knowledge, the first case of Immunoglobulin-associated meningitis described in a patient with Systemic Lupus Erythematosus and hypogammaglobulinemia secondary to Rituximab.Case presentationA 46-year-old female with a past medical history of Systemic Lupus Erythematosus, presented with meningismus 36 h after first infusion of intravenous immunoglobulin. The cerebrospinal fluid analysis showed neutrophilic pleocytosis and hyperproteinorrachia. All microbiological tests were negative. The patient recovered remarkably fast without sequela after just five days of antibiotic therapy.ConclusionSystemic Lupus Erythematosus is a well-documented risk factor for aseptic meningitis associated with other drugs. Possibly, it is also a risk factor for Immunoglobulin associated meningitis. This diagnosis, although rare, should be considered in patients receiving Immunoglobulin since it is a self-limited condition and treatment is supportive.

Atypical Multimetameric Presentation with Paresis of Zoster in an Immunocompromised Patient

We report an uncommon multimetameric presentation (L3, L4 and L5) of zoster with paresis in a 56-year-old-immunocompromised woman with past history of systemic lupus erythematosus. She was treated previously by immunosuppressive therapy with several infectious complications.