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RATIONALE: Limited literatures are available on lower gastrointestinal bleeding in systemic lupus erythematosus (SLE) combined with intestinal tuberculosis. Sharing the treatment experiences of a 26-year-old female patient diagnosed with this complex condition in this report may contribute valuable insights. PATIENT CONCERNS: The patient initially presented with abdominal pain and active gastrointestinal bleeding, leading to admission to the hospital. Over a 2-week period, she experienced persistent bleeding, with daily volumes ranging from 300 mL to 800 mL. DIAGNOSES: Lower gastrointestinal bleeding was diagnosed in this patient with concurrent systemic lupus erythematosus and intestinal tuberculosis. INTERVENTIONS: As her symptoms rapidly progressed, food and water intake had to be completely restricted. The parenteral nutrition was implemented. OUTCOMES: The medical team effectively controlled the bleeding, leading to a notable improvement in the patient's condition. Consequently, she was able to resume oral intake and was discharged from the hospital. LESSONS: This case highlights the significance of using parenteral nutrition in the management of lower gastrointestinal bleeding in patients with concurrent systemic lupus erythematosus and intestinal tuberculosis. Close monitoring and collaborative efforts among healthcare professionals are crucial to achieve successful outcomes in similar cases.
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Enteritis , Lupus Eritematoso Sistémico , Peritonitis Tuberculosa , Tuberculosis Gastrointestinal , Tuberculosis Ganglionar , Humanos , Femenino , Adulto , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/terapia , Lupus Eritematoso Sistémico/diagnóstico , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/terapia , Tuberculosis Gastrointestinal/complicaciones , Tuberculosis Gastrointestinal/diagnóstico , Tuberculosis Gastrointestinal/terapia , Nutrición ParenteralRESUMEN
Objective: This study aimed to investigate the clinical features of angioimmunoblastic T-cell lymphoma (AITL) mimicking systemic lupus erythematosus (SLE) and raise awareness about AITL among rheumatologists in order to prevent misdiagnosis and missed diagnosis. The study reports on a case of AITL mimicking SLE and provides a literature review. Methods: Using key words as search terms, relevant articles published in PubMed before 2022-05 were searched, and their clinical characteristics were collected and analyzed. Results: The literature review retrieved six case reports, including four cases initially diagnosed with SLE and then with AITL. The other two case diagnoses were SLE and AITL, respectively. The two diseases are pathogenically associated and share some common features. The clinical manifestations of AITL are complex. The disease is closely associated with abnormal immune functions and is highly heterogeneous. Conclusion: Patients with AITL generally have a poor prognosis. Rarely do reported cases show AITL mimicking SLE. AITL should be considered during clinical practice to prevent missed diagnoses or misdiagnoses.
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Linfadenopatía Inmunoblástica , Lupus Eritematoso Sistémico , Linfoma de Células T , Humanos , Linfadenopatía Inmunoblástica/diagnóstico , Linfadenopatía Inmunoblástica/complicaciones , Linfadenopatía Inmunoblástica/patología , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/complicaciones , Linfoma de Células T/diagnóstico , Linfoma de Células T/complicaciones , Linfoma de Células T/patologíaRESUMEN
RATIONALE: Systemic lupus erythematosus (SLE) is frequently accompanied by neuropsychiatric (NP) manifestations. However, typical symptoms of catatonia are uncommon. Neuropsychiatric SLE or its "mimickers" may cause NP symptoms, making differential diagnosis a significant challenge in clinical practice. PATIENT CONCERNS: A 68-year-old female with SLE was hospitalized for edema, lung infection, and recurrent fungal mouth ulcers after multiple courses of cortisol and immunosuppressive therapy. Five days after admission, stupor, immobility, mutism, and rigidity were observed. DIAGNOSIS: "Mimickers": catatonic disorder due to a general medical condition. INTERVENTION: Initially, relevant laboratory tests, imaging studies, and the disease activity index score were performed. A survey of the causes of the disease was conducted among the patient's relatives. Subsequently, we discontinued moxifloxacin, corticosteroids, fluconazole, and other medications and inserted a gastric tube for nutritional support. During this process, traditional Chinese medicine and acupuncture have been utilized. OUTCOMES: After 3 days, the patient recovered and only complained of fatigue. CONCLUSION: When SLE presents with NP symptoms, it is essential to make a correct diagnosis in order to guide appropriate treatment by actively searching for inducers and clinical, laboratory, and neuroradiological characteristics that can aid in the differential diagnosis. When treatment options are limited, it can be beneficial to try a variety of combination strategies, such as traditional Chinese medicine and acupuncture.
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Catatonia , Lupus Eritematoso Sistémico , Vasculitis por Lupus del Sistema Nervioso Central , Trastornos Mentales , Femenino , Humanos , Anciano , Catatonia/diagnóstico , Catatonia/etiología , Catatonia/terapia , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Vasculitis por Lupus del Sistema Nervioso Central/diagnóstico , Trastornos Mentales/diagnóstico , Corticoesteroides/uso terapéuticoRESUMEN
BACKGROUND: The clinical presentation of mercury (Hg) intoxication may mimic rheumatic diseases. Hg exposure is associated with systemic lupus erythematosus (SLE)-like disease in genetically susceptible rodents and Hg is among the environmental factors in the development of SLE in humans. Herein, we presented a case with clinical and immunological features suggestive of SLE but diagnosed with Hg intoxication. CASE: A thirteen-year-old female with myalgia, weight loss, hypertension and proteinuria was referred to our clinic for the evaluation of possible SLE. Physical examination of the patient was unremarkable except for a cachectic appearance and hypertension, laboratory investigation revealed positive anti-nuclear antibody, dsDNA antibody and hypocomplementemia with nephrotic range proteinuria. Inquiry for toxic exposures revealed a continuous exposure to an unknown silverly shiny liquid for a month which was thought to be Hg. Due to the fulfillment of Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE, a percutaneous kidney biopsy was performed whether proteinuria resulted because of the Hg exposure or flare of lupus nephritis. Blood and 24-hour urine Hg levels were high, and no findings associated with SLE were observed in the examination of the kidney biopsy. The patient was diagnosed with Hg intoxication and, clinical and laboratory findings, including hypocomplementemia, positive ANA and anti-dsDNA antibody, improved with chelation therapy. Also, no findings associated with SLE were observed in the follow-up of the patient. CONCLUSIONS: In addition to the toxic effects, Hg exposure may cause autoimmune features. As far as we know, this is the first-time Hg exposure was associated with hypocomplementemia and anti-dsDNA antibody in a patient. Also, this case highlights the inconvenience of the use of classification criteria for diagnostic purposes.
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Hipertensión , Lupus Eritematoso Sistémico , Mercurio , Femenino , Humanos , Adolescente , Lupus Eritematoso Sistémico/inducido químicamente , Lupus Eritematoso Sistémico/diagnóstico , Terapia por Quelación , ProteinuriaRESUMEN
We report an 11-year-old girl with systemic lupus erythematosus (SLE) who showed hypophosphataemia (1.7 mg/dl, normal range: 3.9-5.8 mg/dl), a decrease in the tubular maximum reabsorption of phosphate/glomerular filtration rate (TmP/GFR) (0.77 mg/dl, normal range: 3.4-5.6 mg/dl), and an elevated serum fibroblast growth factor 23 (FGF23) (circulating phosphate-regulatory hormone) concentration (FGF23: 282 pg/ml, normal range: <52 pg/ml) at the onset. The patient was treated with intravenous pulse methylprednisolone, oral prednisolone, mycophenolate mofetil, hydroxychloroquine, and phosphorus supplement. Serum FGF23 concentrations decreased to near the reference value at 5 months after the onset of SLE, and the TmP/GFR (4.61 mg/dl) simultaneously improved. The urinary deoxypyridinoline (bone resorption marker) concentration on admission (18.9 nmol/mmol creatinine, normal range: 75.4 ± 6.8 nmol/mmol creatinine) was greatly reduced, and the bone-type alkaline phosphatase (bone formation marker) concentration (30.6 µg/l, normal range: 58.6 ± 15.3 µg/l) was also reduced during the increase in FGF23 concentrations before steroid therapy was initiated. The reason for the inappropriate secretion of FGF23, despite hypophosphataemia, remains unknown. The findings in our case suggest that changes in bone turnover markers can occur in patients with SLE and excess inappropriate secretion of FGF23, despite severe and persistent hypophosphataemia.
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Hipofosfatemia , Lupus Eritematoso Sistémico , Femenino , Humanos , Niño , Factor-23 de Crecimiento de Fibroblastos , Creatinina , Factores de Crecimiento de Fibroblastos/metabolismo , Hipofosfatemia/etiología , Fosfatos/metabolismo , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Remodelación ÓseaRESUMEN
INTRODUCTION: In this study, it was aimed to compare the effects of both melatonin and 25-hydroxyvitamin D3, defined as an immune modulator, on laboratory diagnostic criteria parameters and disease activity in patients with systemic lupus erythematosus (SLE). METHODS: The study included 56 women with SLE and 40 healthy women (control group). Melatonin and 25-hydroxyvitamin D3 levels of patients and healthy individuals included in the study were examined. In addition, leukocytes, lymphocytes, platelets, C3, C4, anti-double-stranded DNA (Anti-dsDNA), antinuclear antibody, and SLE disease activity index (SLEDAI) were analyzed in women with SLE. Patients were divided into four subgroups according to SLEDAI. RESULTS: Melatonin and 25-hydroxyvitamin D3 levels of women with SLE were lower than healthy women (p < 0.001). Both melatonin and 25-hydroxyvitamin D3 levels were not correlated with laboratory diagnostic criteria parameters. Only 25-hydroxyvitamin D3 levels were correlated with leukocyte levels (p < 0.01). There was no significant difference between the melatonin levels of the subgroups. The 25-hydroxyvitamin D3 levels of the subgroup without disease activity were higher than levels of the subgroups with disease activity (p < 0.05). There was a negative correlation between SLEDAI score and 25-hydroxyvitamin D3 levels (p < 0.05). CONCLUSION: Women with SLE had lower melatonin and 25-hydroxyvitamin D3 levels than healthy women. On the other hand, parameters of laboratory diagnostic criteria of SLE disease were not related. Only 25-hydroxyvitamin D3 levels were inversely related leukocyte levels. SLE disease activity was not correlated with melatonin levels but negatively correlated with 25-hydroxyvitamin D3 levels. Key Points ⢠Women with SLE have low levels of melatonin and 25-hydroxyvitamin D3. ⢠Melatonin and 25-hydroxyvitamin D3 levels are not related to the laboratory diagnostic criteria parameters for SLE disease. ⢠Low levels of melatonin and 25-hydroxyvitamin D3 may be a factor in the unbalanced immune system of SLE. ⢠Supplementation of melatonin and 25-hydroxyvitamin D3 may be recommended for women patients with SLE.
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Lupus Eritematoso Sistémico , Melatonina , Humanos , Femenino , Calcifediol , Oscuridad , Lupus Eritematoso Sistémico/diagnóstico , Anticuerpos AntinuclearesRESUMEN
BACKGROUND: Transepidermal clearance of altered collagen and excessive excretion of keratin are characteristics of a rare cutaneous disorder known as reactive perforating collagenosis (RPC). There are different forms of RPC; however, the acquired form is the most prevalent and inherited. Reactive perforating collagenosis is rarely described in autoimmune rheumatic diseases; instead, it is typically linked to systemic conditions such as renal failure or hepatic disease. METHODS: A 31-year-old Saudi female patient who was initially diagnosed with undifferentiated connective tissue disease. She developed RPC with a severe diffuse itchy skin rash with numerous papules and nodules with central hyperkeratotic plugs over the lower limb, upper limb, and face. RESULTS: The patient tested positive for antinuclear antibody; however, a year later, patient developed Raynaud's phenomenon, oral and nasal ulcers, malar rash, fatigue, and lupus rash around her eyes, and systemic lupus erythematosus was diagnosed clinically. The patient was treated for reactive perforating collagenosis with systemic antihistamines (diphenhydramine 50 mg orally twice daily), topical steroid cream (betamethasone dipropionate cream), and oral isotretinoin (20 mg daily). The patient was advised to undergo phototherapy. A year later, she presented with symptoms of systemic lupus erythematosus and started taking oral hydroxychloroquine 200 mg twice daily for systemic lupus erythematosus. The patient is listed on follow-up. CONCLUSION: Variable skin rash can mimic systemic lupus erythematosus and vasculitis. Therefore, reactive perforating collagenosis is a skin condition that requires high clinical suspension for diagnosis, and it might be challenging to determine whether it is an association or a complication. Furthermore, the timing of the skin biopsy may be crucial for the diagnosis of reactive perforating collagenosis.
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Exantema , Lupus Eritematoso Sistémico , Femenino , Humanos , Adulto , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnósticoRESUMEN
The autoimmune/inflammatory syndrome induced by adjuvants (ASIA), also known as Shoenfeld's syndrome, encompasses several autoimmune conditions/phenomena that are triggered following the exposure to materials with an adjuvant activity known to augment an antigen-driven immune response. In some inherently vulnerable patients, they act as second hits to trigger or unmask an autoimmune disorder which ranges from generalized non-specific constitutional symptoms, and autoantibody production, to a new onset, of a fully-fledged autoimmune syndrome. In this manuscript, we present a case of a 37-year-old lady who developed systemic lupus erythematosus characterized by mucocutaneous, musculoskeletal, hematological neurological, and renal involvement a few years after silicone breast implants.
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Enfermedades Autoinmunes , Implantes de Mama , Lupus Eritematoso Sistémico , Adyuvantes Inmunológicos/efectos adversos , Adulto , Femenino , Humanos , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Siliconas/efectos adversos , SíndromeRESUMEN
To explore the effect of atorvastatin combined with Zishen Qingqi granules on the immune function and liver function of patients with mild to moderate activity systemic lupus erythematosus. The data of 120 patients with mild to moderate activity systemic lupus erythematosus admitted to our hospital from February 2019 to February 2020 were retrospectively analyzed and they were divided into experimental group (n=60) and the control group (n=60) according to the order of admission; the control group was treated with atorvastatin, and the experimental group was treated with Zishen Qingqi granules plus. The immune function, liver function, TCM syndrome score and systemic lupus erythematosus disease activity index (SLEDAI) were compared between the two groups. The experimental group after treatment was superior to the control group with respect to immune function indexes, liver function indexes, SLEDAI and TCM syndromes (all P<0.001). Atorvastatin combined with Zishen Qingqi granules can improve the liver function of patients with mild to moderate activity systemic lupus erythematosus, enhance their immunity, and relieve their clinical symptoms.
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Atorvastatina/uso terapéutico , Medicamentos Herbarios Chinos/uso terapéutico , Inhibidores de Hidroximetilglutaril-CoA Reductasas/uso terapéutico , Hígado/efectos de los fármacos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Adulto , Atorvastatina/efectos adversos , Quimioterapia Combinada , Medicamentos Herbarios Chinos/efectos adversos , Femenino , Humanos , Inhibidores de Hidroximetilglutaril-CoA Reductasas/efectos adversos , Hígado/metabolismo , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/inmunología , Lupus Eritematoso Sistémico/metabolismo , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: The efficacy and safety of integrated Chinese and western medicine in the treatment of Systemic Lupus Erythematosus (SLE) have not been systematically evaluated. METHODS: This systematic review and meta-analysis will be performed and reported according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We will search PubMed, Embase, Cochrane Library, Chinese Biomedical Database WangFang, VIP, and China National Knowledge Infrastructure (CNKI) for potentially eligible studies from their inception to Dec. 2020, and we will select all the eligible randomized controlled trials (RCTs) of the integrated Chinese and western medicine for Systemic Lupus Erythematosus. All the studies will be screened according to the inclusion and exclusion criteria and meta-analysis will be conducted using Review Manager V.5.3. and Stata V.12.0. The primary endpoint was the clinical efficacy rate, the secondary outcomes were SLEDAI score, adverse reaction rate, and laboratory test parameters (white blood cells, complement C3). RESULTS: The results of this study will systematically evaluate the effectiveness and safety of the integrated Chinese and western medicine in the treatment of primary dysmenorrhea. CONCLUSION: The efficacy and safety of integrated Chinese and western medicine will be systematically evaluated in this paper, thus providing evidence for the clinical application of this therapy. ETHICS AND DISSEMINATION: This study is a systematic review, which is based on the published studies, so examination and agreement by the ethics committee are not required in this study. We intend to publish the study results in a journal or conference presentations. OPEN SCIENCE FRAMEWORK OSFREGISTRATION NUMBER: https://osf.io/mabxh.
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Medicamentos Herbarios Chinos/uso terapéutico , Glucocorticoides/uso terapéutico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Quimioterapia Combinada/métodos , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Metaanálisis como Asunto , Ensayos Clínicos Controlados Aleatorios como Asunto , Índice de Severidad de la Enfermedad , Revisiones Sistemáticas como Asunto , Resultado del TratamientoRESUMEN
BACKGROUND: A previous study have evaluated that antinuclear antibodies (ANA) negativization is linked to low lupus disease activity. AIM: To describe a lupus patient who evolved with negativization of ANA, anti-dsDNA, and anti-chromatin antibodies after vitamin D supplementation. METHOD: Case report. RESULTS: A 56-year-old female patient, diagnosed with systemic lupus erythematosus since 2015 characterized by typical malar erythema, photosensitivity, polyarthritis, leucopenia, positive antinuclear antibody, anti-dsDNA, and anti-chromatin antibody. She received hydroxychloroquine and prednisone. After 1 year, corticotherapy was tapered off, and no clinical evidence of lupus activity was registered (SLEDAI = 0). However, ANA remained positive with a titer of 1:640 with a homogeneous pattern, and positive anti-dsDNA 1/20 and anti-chromatin 97 Units (normal range: <20 Units) remained all-time positive. Treatment with vitamin D 25,000 IU/day was initiated, and during follow-up, anti-chromatin and anti-dsDNA disappeared. In 2019, the patient was asymptomatic, keeping SLEDAI = 0, negative anti-dsDNA and anti-chromatin, and surprisingly the ANA turned negative, which was confirmed on several occasions until now. CONCLUSION: This case adds knowledge to the understanding that negative antinuclear antibodies appear to be associated with a better prognosis in lupus patients. Furthermore, the use of vitamin D seems to be a complementary therapeutic tool for this purpose.
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Anticuerpos Antinucleares , Lupus Eritematoso Sistémico , Cromatina , Suplementos Dietéticos , Femenino , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Persona de Mediana Edad , Vitamina DRESUMEN
Lupus is a systemic autoimmune disease typified by uncontrolled inflammation, disruption of immune tolerance, and intermittent flaring - events triggerable by environmental factors. Preclinical and clinical studies reveal that consumption of the marine ω-3 highly unsaturated fatty acids (HUFAs) eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) might be used as a precision nutrition intervention to lessen lupus symptoms. The anti-inflammatory and pro-resolving effects of ω-3 HUFAs are inextricably linked to their presence in membrane phospholipids. The ω-3 HUFA score, calculated as [100 × (ω-3 HUFAs/(ω-3 HUFAs + ω-6 HUFAs))] in red blood cells (RBCs), and the Omega-3 Index (O3I), calculated as [100 × ((DHA+EPA)/total fatty acids)] in RBCs, are two biomarkers potentially amenable to relating tissue HUFA balance to clinical outcomes in individuals with lupus. Using data from three prior preclinical DHA supplementation studies, we tested the hypothesis that the ω-3 HUFA score and the O3I inversely correlate with indicators of autoimmune pathogenesis in the cSiO2-triggered lupus flaring model. The three studies employed both low and high fat rodent diets, as well as more complex diets emulating the U.S. dietary pattern. The ω-3 HUFA scores in RBCs were comparatively more robust than the O3I at predicting HUFA balances in the kidney, liver, spleen, and lung. Importantly, increases in both the ω-3 HUFA score (>40%) and the O3I (>10%) were strongly associated with suppression of cSiO2-triggered (1) expression of interferon-regulated genes, proinflammatory cytokine production, leukocyte infiltration, and ectopic lymphoid structure development in the lung, (2) pulmonary and systemic autoantibody production, and (3) glomerulonephritis. Collectively, these findings identify achievable ω-3 HUFA scores and O3I thresholds that could be targeted in future human intervention studies querying how ω-3 HUFA consumption influences lupus and other autoimmune diseases.
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Eritrocitos/metabolismo , Ácidos Grasos Omega-3/sangre , Ácidos Grasos Omega-6/sangre , Lupus Eritematoso Sistémico/sangre , Alimentación Animal , Animales , Autoinmunidad , Biomarcadores/sangre , Líquido del Lavado Bronquioalveolar/inmunología , Citocinas/metabolismo , Dieta , Modelos Animales de Enfermedad , Ácidos Grasos Omega-3/administración & dosificación , Ácidos Grasos Omega-6/administración & dosificación , Femenino , Mediadores de Inflamación/metabolismo , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/dietoterapia , Lupus Eritematoso Sistémico/inmunología , Ratones Endogámicos NZB , Valor Predictivo de las Pruebas , Brote de los SíntomasAsunto(s)
Antiinflamatorios/uso terapéutico , Edema/diagnóstico , Glucocorticoides/uso terapéutico , Lupus Eritematoso Sistémico/diagnóstico , Metilprednisolona/uso terapéutico , Edad de Inicio , Niño , Diagnóstico Diferencial , Edema/tratamiento farmacológico , Edema/patología , Exantema/diagnóstico , Exantema/tratamiento farmacológico , Exantema/patología , Femenino , Fiebre/diagnóstico , Fiebre/tratamiento farmacológico , Fiebre/patología , Humanos , Lupus Eritematoso Sistémico/tratamiento farmacológico , Lupus Eritematoso Sistémico/patología , Órbita/efectos de los fármacos , Órbita/patología , Faringitis/diagnóstico , Faringitis/tratamiento farmacológico , Faringitis/patología , Resultado del TratamientoRESUMEN
An 8-year-old girl with recently diagnosed Systemic Lupus Erythematosus (SLE) (class 4 lupus nephritis with autoimmune hemolytic anemia) presented to the pediatric nephrology clinic with polyuria, tiredness and cramps; laboratory investigations revealed refractory hypokalemia, hypomagnesemia, metabolic alkalosis, hypocalciuria and hyperchloriuria. There was no history of diuretic administration. These features were consistent with the Gitelman syndrome. She required large doses of potassium and magnesium supplementation along with spironolactone, for normalization of the serum potassium and magnesium levels. Immunosuppressive therapy was continued with cyclophosphamide pulses administered on a monthly basis. The doses of potassium and magnesium supplements were tapered off over the next 6 months. The clinical exome sequencing was negative for any mutations in the SLC12A3 gene. An 'acquired' form of Gitelman syndrome has been reported earlier in association with Sjogren syndrome and systemic sclerosis. Though tubular disorders such as renal tubular acidosis have been reported in association with SLE, a Gitelman-like syndrome has not been reported earlier. This case adds Gitelman-like tubulopathy to the clinical spectrum of tubular disorders complicating SLE.
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Síndrome de Gitelman/etiología , Lupus Eritematoso Sistémico/complicaciones , Nefritis Lúpica/complicaciones , Poliuria/diagnóstico , Alcalosis/diagnóstico , Niño , Ciclofosfamida/administración & dosificación , Ciclofosfamida/efectos adversos , Ciclofosfamida/uso terapéutico , Quimioterapia Combinada , Femenino , Síndrome de Gitelman/diagnóstico , Síndrome de Gitelman/tratamiento farmacológico , Síndrome de Gitelman/orina , Humanos , Hipopotasemia/diagnóstico , Hipopotasemia/tratamiento farmacológico , Inmunosupresores/administración & dosificación , Inmunosupresores/efectos adversos , Inmunosupresores/uso terapéutico , Túbulos Renales/patología , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/tratamiento farmacológico , Nefritis Lúpica/diagnóstico , Nefritis Lúpica/patología , Magnesio/administración & dosificación , Magnesio/uso terapéutico , Antagonistas de Receptores de Mineralocorticoides/administración & dosificación , Antagonistas de Receptores de Mineralocorticoides/uso terapéutico , Poliuria/etiología , Potasio/administración & dosificación , Potasio/uso terapéutico , Inducción de Remisión , Espironolactona/administración & dosificación , Espironolactona/uso terapéuticoRESUMEN
BACKGROUND: Recent evidence has suggested that the 1,25(OH)2D3/Vitamin D receptor (VDR) acts to suppress the immune response associated with systemic lupus erythematosus (SLE), a serious multisystem autoimmune disease. Hence, the aim of the current study was to investigate the mechanism by which 1,25-(OH)2D3/VDR influences SLE through regulating the Skp2/p27 signaling pathway. METHODS: Initially, the levels of 1,25(OH)2D3, VDR, Skp2, and p27 were measured in collected renal tissues and peripheral blood. Meanwhile, the levels of inflammatory factors, biochemical indicators (BUN, Cr, anti-nRNP IgG, anti-dsDNA IgG) and urinary protein levels were assayed in in VDRinsert and VDR-knockout mice in response to 1,25(OH)2D3 supplement. In addition, the distribution of splenic immune cells was observed in these mice. RESULTS: Among the SLE patients, the levels of 1,25(OH)2D3, VDR and p27 were reduced, while the levels of Skp2 were elevated. In addition, the levels of anti-nRNP IgG and anti-dsDNA IgG were increased, suggesting induction of inflammatory responses. Notably, 1,25(OH)2D3/VDR mice had lower concentrations of BUN and Cr, urinary protein levels, precipitation intensity of the immune complex and complement, as well as the levels of anti-nRNP IgG and anti-dsDNA IgG in SLE mice. Additionally, 1,25(OH)2D3 or VDR reduced the degree of the inflammatory response while acting to regulate the distribution of splenic immune cells. CONCLUSION: This study indicated that 1,25-(OH)2D3/VDR facilitated the recovery of SLE by downregulating Skp2 and upregulating p27 expression, suggesting the potential of 1,25-(OH)2D3/VDR as a promising target for SLE treatment.
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Calcitriol/metabolismo , Inhibidor p27 de las Quinasas Dependientes de la Ciclina/metabolismo , Lupus Eritematoso Sistémico/metabolismo , Receptores de Calcitriol/metabolismo , Proteínas Quinasas Asociadas a Fase-S/metabolismo , Adolescente , Adulto , Anciano , Animales , Calcitriol/administración & dosificación , Calcitriol/análisis , Niño , Inhibidor p27 de las Quinasas Dependientes de la Ciclina/análisis , Suplementos Dietéticos , Regulación hacia Abajo , Femenino , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Masculino , Ratones , Ratones Endogámicos C57BL , Ratones Noqueados , Persona de Mediana Edad , Receptores de Calcitriol/análisis , Receptores de Calcitriol/deficiencia , Proteínas Quinasas Asociadas a Fase-S/análisis , Transducción de Señal , Regulación hacia Arriba , Adulto JovenRESUMEN
BACKGROUND: Systemic lupus erythematosus (SLE or lupus) is a chronic autoimmune disease that is associated with increased morbidity, mortality, healthcare costs and decreased quality of life. African Americans in the USA have three to four times greater prevalence of SLE, risk of developing SLE at an earlier age, and SLE-related disease activity, damage, and mortality compared with Caucasians, with the highest rates experienced by African American women. There is strong evidence that patient-level factors are associated with outcomes, which justifies targeting them with intervention. While evidence-based self-management interventions that incorporate both social support and health education have reduced pain, improved function, and delayed disability among patients with SLE, African Americans and women are still disproportionately impacted by SLE. Peer mentoring interventions are effective in other chronic conditions that disproportionately affect minorities, such as diabetes mellitus, HIV, and kidney disease, but there is currently no empirically tested peer mentoring intervention developed for patients with SLE. Preliminary data from our group suggest that peer mentoring improves self-management, reduces disease activity, and improves health-related quality of life (HRQOL) in African American women with SLE. METHODS: This study will test an innovative, manualized peer mentorship program designed to provide modeling and reinforcement by peers (mentors) to other African American women with SLE (mentees) to encourage them to engage in activities that promote disease self-management. Through a randomized, "mentored" or "support group" controlled design, we will assess the efficacy and mechanism(s) of this intervention in self-management, disease activity, and HRQOL. DISCUSSION: This is the first study to test peer mentorship as an alternative strategy to improve outcomes in African American women with SLE. This could result in a model for other programs that aim to improve disease self-management, disease activity, and HRQOL in African American women suffering from chronic illness. The peer mentoring approach is uniquely fitted to African Americans, and this intervention has the potential to lead to health improvements for African American women with SLE that have not been attainable with other interventions. This would significantly reduce disparities and have considerable public health impact. TRIAL REGISTRATION: ClinicalTrials.gov, NCT03734055 . Registered on 27 November 2018.
Asunto(s)
Negro o Afroamericano/psicología , Conductas Relacionadas con la Salud/etnología , Conocimientos, Actitudes y Práctica en Salud/etnología , Lupus Eritematoso Sistémico/terapia , Tutoría , Influencia de los Compañeros , Automanejo , Femenino , Humanos , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/etnología , Lupus Eritematoso Sistémico/psicología , Calidad de Vida , Ensayos Clínicos Controlados Aleatorios como Asunto , Factores Sexuales , Factores de Tiempo , Resultado del TratamientoRESUMEN
BACKGROUND: No population-based study has investigated the cumulative incidence of pulmonary arterial hypertension (PAH) in patients with newly diagnosed systemic lupus erythematosus (SLE) or the survival impact of PAH in this population. METHOD: We used a nationwide database in Taiwan and enrolled incident SLE patients between January 1, 2000, and December 31, 2013. The cumulative incidence of PAH in the SLE patients and the survival of these patients were estimated by the Kaplan-Meier method. Potential predictors of the development of PAH were determined using a Cox proportional hazards regression model. RESULTS: Of 15,783 SLE patients, 336 (2.13%) developed PAH. The average interval from SLE diagnosis to PAH diagnosis was 3.66 years (standard deviation [SD] 3.36, range 0.1 to 13.0 years). Seventy percent of the patients developed PAH within 5 years after SLE onset. The 3- and 5-year cumulative incidence of PAH were 1.2% and 1.8%, respectively. Systemic hypertension was an independent predictor of PAH occurrence among the SLE patients (adjusted hazard ratio 2.27, 95% confidence interval 1.59-2.97). The 1-, 3-, and 5-year survival rates of SLE patients following the diagnosis of PAH were 87.7%, 76.8%, and 70.1%, respectively. CONCLUSIONS: PAH is a rare complication of SLE and the majority of PAH cases occur within the first 5 years following SLE diagnosis. Systemic hypertension may be a risk factor for PAH development in the SLE population. The overall 5-year survival rate after PAH diagnosis was 70.1%.
Asunto(s)
Hipertensión Pulmonar/epidemiología , Lupus Eritematoso Sistémico/epidemiología , Vigilancia de la Población/métodos , Hipertensión Arterial Pulmonar/epidemiología , Adulto , Anciano , Estudios de Cohortes , Comorbilidad , Femenino , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/mortalidad , Incidencia , Estimación de Kaplan-Meier , Lupus Eritematoso Sistémico/diagnóstico , Lupus Eritematoso Sistémico/mortalidad , Masculino , Persona de Mediana Edad , Programas Nacionales de Salud/estadística & datos numéricos , Modelos de Riesgos Proporcionales , Hipertensión Arterial Pulmonar/diagnóstico , Hipertensión Arterial Pulmonar/mortalidad , Tasa de Supervivencia , Taiwán/epidemiologíaRESUMEN
OBJECTIVE: To compare the quality of care received by patients with systemic lupus erythematosus (SLE) in 2 settings within the academic institution (a dedicated lupus clinic and a general rheumatology clinic) using validated SLE quality measures. METHODS: One hundred fifty consenting, consecutive SLE patients receiving longitudinal care at the Rush University general rheumatology clinic (n = 73) or the subspecialty lupus clinic (n = 77) were recruited. An updated quality measure survey and retrospective medical chart review were used to evaluate each quality measure (n = 20). The overall and individual quality measure performance was calculated and compared between the 2 groups. Data on the number of SLE patients seen by each rheumatologist were collected to assess the relationship between SLE patient volume and quality measures. RESULTS: Overall quality measure performance was significantly better in SLE patients receiving care at the lupus clinic (85.8% versus 70.2% of patients receiving care at the general rheumatology clinic; P = 0.001). Differences between the 2 groups were observed for sunscreen counseling (98.7% and 83.6%, respectively; P = 0.001), antiphospholipid antibody testing (71.4% and 37%, respectively; P < 0.001), pneumococcal vaccination (84.8% and 48.8%, respectively; P < 0.001), bone mineral density testing (94.2% and 54.5%, respectively; P < 0.001), drug counseling (92.2% and 80.8%, respectively; P = 0.04), use of a steroid-sparing agent (100% and 82%, respectively; P < 0.007), use of an angiotensin-converting enzyme inhibitor (94.4% and 58.3%, respectively; P = 0.03), and cardiovascular disease risk assessment (40.3% and 15.1%, respectively; P = 0.01). There was a moderate correlation between physician volume and quality measure performance (ρ = 0.48, P < 0.001). CONCLUSION: Compared with the general rheumatology clinic, the dedicated lupus clinic had better quality measure performance in this cross-sectional single-center study. In our health care system, we also observed indicators suggesting that rheumatologists with a higher volume of SLE patients provide higher quality of care.