Risk factors associated with the death of patients hospitalized for juvenile systemic lupus erythematosus

Facó, Mércia Maria Moreira; Leone, Cláudio; Campos, Lúcia Maria Arruda; Febrônio, Marília Vieira; Marques, Heloisa; Silva, C.A.

doi:10.1590/s0100-879x2006005000110

Cited by 70 publications

(82 citation statements)

References 36 publications

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“…Childhood-onset systemic lupus erythematosus (cSLE) is a rare autoimmune disorder that may affect multiple organs and systems [1,2] . The mucocutaneous involvement has been reported in approximately 70% of these patients [1,3,4] .…”

Section: Introductionmentioning

confidence: 99%

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Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

Ferriani

Silva

Pereira

et al. 2015

Int Arch Allergy Immunol

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Background: Data regarding the prevalence of chronic spontaneous urticaria (CSU) in childhood-onset systemic lupus erythematosus (cSLE) patients and possible associated factors are limited to a few case reports. The objectives of this study were to assess CSU in a large cSLE population, in order to evaluate the demographic data, clinical manifestations, disease activity/damage, laboratory abnormalities and treatment. Methods: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services and included 852 cSLE patients. CSU was diagnosed according to the guidelines of the European Academy of Allergy and Clinical Immunology, the Global Allergy and Asthma European Network, the European Dermatology Forum and the World Allergy Organization. Patients with CSU (evaluated at urticaria diagnosis) and patients without CSU (evaluated at the last visit) were assessed for lupus clinical/laboratory features and treatment. Results: CSU was observed in 10/852 cSLE patients (1.17%). The median of cSLE duration at urticaria diagnosis was 0 (-3 to 5) years. Comparison of cSLE patients with and without CSU revealed a greater frequency of constitutional symptoms (40 vs. 8%, p = 0.006), reticuloendothelial system involvement (30 vs. 3%, p = 0.003), mucocutaneous (90 vs. 28%, p < 0.0001) and musculoskeletal manifestations (50 vs. 6%, p < 0.0001) and methylprednisolone pulse therapy use (60 vs. 9%, p < 0.0001) in the former group. The frequency of immunosuppressive treatment was lower in patients with CSU (p = 0.018). The median SLE Disease Activity Index 2000 (12 vs. 2, p < 0.0001) and erythrocyte sedimentation rate (40 vs. 19 mm/1st hour, p = 0.024), was higher in patients with CSU. Conclusions: To our knowledge, this is the first study with evidence that CSU may be linked to cSLE. We also demonstrated that this particular skin manifestation occurs predominantly at disease onset and is associated with lupus moderate/high disease activity without major organ involvement.

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Section: Introductionmentioning

confidence: 99%

“…Indeed, a variety of cutaneous manifestations can be observed in lupus patients [1,3,4] , including urticaria [5][6][7][8] . This skin involvement is defined by the sudden onset of wheals, angioedema or both.…”

Section: Introductionmentioning

confidence: 99%

Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

Ferriani

Silva

Pereira

et al. 2015

Int Arch Allergy Immunol

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“…1 Esta miopatia infecciosa está associada a condiç ões que levam à imunossupressão, especialmente a infecção pelo vírus da imunodeficiência humana (HIV), o diabetes mellitus, a malignidade, os fármacos imunossupressores e as doenças reumáticas. [1][2][3][4][5][6][7] As infecç ões são uma das principais causas de morbidade e mortalidade em pacientes com lúpus eritematoso sistêmico (LES) e lúpus eritematoso sistêmico juvenil (LESJ), 8,9 podendo envolver a musculatura esquelética. A piomiosite é raramente relatada em adultos com LES, 3-7 e, até onde se sabe, ainda não foi diagnosticada em pacientes com LESJ.…”

Section: Introductionunclassified

Piomiosite no lúpus eritematoso sistêmico juvenil

Blay

Ferriani²,

Buscatti

et al. 2016

Revista Brasileira de Reumatologia

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Pyomyositis is a pyogenic infection of skeletal muscle that arises from hematogenous spread and usually presents with localized abscess. This muscle infection has been rarely reported in adult-onset systemic lupus erythematous and, to the best of our knowledge, has not been diagnosed in pediatric lupus population. Among our childhood-onset systemic lupus erythematous population, including 289 patients, one presented pyomyositis. This patient was diagnosed with childhood-onset systemic lupus erythematous at the age of 10 years-old. After six years, while being treated with prednisone, azathioprine and hydroxychloroquine, she was hospitalized due to a 30-day history of insidious pain in the left thigh and no apparent trauma or fever were reported. Her physical examination showed muscle tenderness and woody induration. Laboratory tests revealed anemia, increased acute phase reactants and normal muscle enzymes. Computer tomography of the left thigh showed collection on the middle third of the vastus intermedius, suggesting purulent stage of pyomyositis. Treatment with broad-spectrum antibiotic was initiated, leading to a complete clinical resolution. In conclusion, we described the first case of pyomyositis during childhood in pediatric lupus population. This report reinforces that the presence of localized muscle pain in immunocompromised patients, even without elevation of muscle enzymes, should raise the suspicion of pyomyositis. A prompt antibiotic therapy is strongly recommended.

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“…4,5 A inflamação dos vasos sanguíneos é uma manifestação comum da doença e pode afetar vasos de pequeno, médio e grande porte. [4][5][6][7] Além disso, o LES-i e a PANS-i podem apresentar manifestaç ões clínicas e alteraç ões laboratoriais semelhantes. No entanto, que se tem conhecimento, a síndrome de sobreposição dessas duas doenças ainda não foi descrita.…”

Section: Introductionunclassified

Poliartrite nodosa sistêmica e lúpus eritematoso sistêmico de início na infância: uma síndrome de sobreposição?

Marques

Guariento

Simões

et al. 2016

Revista Brasileira de Reumatologia

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We described herein a patient who presented an overlap syndrome of childhood-onset systemic polyarteritis nodosa (c-PAN) and childhood-onset systemic lupus erythematosus (c-SLE). A 9-year-old girl presented tender subcutaneous nodules on feet, arterial hypertension, right hemiplegia and dysarthric speech. She was hospitalized due to stroke and left foot drop. Brain computer tomography showed ischemic stroke. Magnetic resonance angiography revealed stenosis in the middle cerebral and internal carotid arteries. Electroneuromyography identified a mononeuropathy of left posterior tibial nerve and she fulfilled the c-PAN validated criteria. She was treated with intravenous methylprednisolone pulse therapy followed by prednisone, that was progressively tapered, six months of intravenous cyclophosphamide and after that she received azathioprine for 19 months. At the age of 14 years and 9 months, she presented malar rash, photosensitivity, edema in lower limbs and arterial hypertension. The proteinuria was 1.7g/day. Antinuclear antibodies (ANA) were 1/1280 (homogeneous nuclear pattern) and anti-dsDNA antibodies were positive. Renal biopsy showed focal proliferative and membranous glomerulonephritis. Therefore, she fulfilled the American College of Rheumatology classification criteria for SLE and she was treated with prednisone, hydroxychloroquine and mycophenolate mofetil. In conclusion, we described herein a possible overlap syndrome of two autoimmune diseases, where c-PAN occurred five years before the c-SLE diagnosis.

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Risk factors associated with the death of patients hospitalized for juvenile systemic lupus erythematosus

Cited by 70 publications

References 36 publications

Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus

Piomiosite no lúpus eritematoso sistêmico juvenil

Poliartrite nodosa sistêmica e lúpus eritematoso sistêmico de início na infância: uma síndrome de sobreposição?

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