A novel therapeutic approach using the Zipper method to treat chorea in a pediatric-onset systemic lupus erythematosus patient

Lupus ◽  
2021 ◽  
pp. 096120332098401
Author(s):  
Esra Baglan ◽  
Semanur Ozdel ◽  
Mutlu Uysal Yazıcı ◽  
Ebru Azapağası ◽  
Halil Çelik ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Immunosuppressive Therapy ◽  
Lupus Erythematosus ◽  
Central Nervous System Involvement ◽  
Clinical Findings ◽  
Systemic Lupus ◽  
First Case ◽  
Neuropsychiatric Sle ◽  
Pediatric Onset ◽  
Onset Systemic Lupus Erythematosus

Pediatric-onset systemic lupus erythematosus is among the prototypic systemic autoimmune diseases seen in children. Although the neuropsychiatric involvement rate varies during the course of the disease, it is an important cause of morbidity and mortality. The clinical picture of neuropsychiatric SLE (NPSLE) is highly variable, and neurological features can precede systemic findings, leading to some diagnostic difficulties. NPSLE requires early and aggressive immunosuppressive therapy. Some patients can be resistant to immunosuppressive therapy. Chorea is a rare manifestation that occurs in 1.2%–2% of SLE patients and can result from an immunologically mediated mechanism, antiphospholipid autoantibodies or ischemia. Herein we present the first case of pediatric-onset SLE diagnosed with central nervous system involvement and treated with Zipper method. The Zipper method is a new immunomodulation treatment. The clinical findings of the patient, which were resistant to corticosteroids and cyclophosphamide, resolved by this novel treatment.

scholarly journals Stercoral Colitis in a Patient With Pediatric-Onset Systemic Lupus Erythematosus: Case Analysis and Review of the Literature

2021 ◽  
Vol 9 ◽  
Author(s):  
Chun-Chun Gau ◽  
Li-Lun Lin ◽  
Chao-Yi Wu ◽  
Jing-Long Huang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Abdominal Pain ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Rare Type ◽  
Multiple Organs ◽  
First Case ◽  
Neuropsychiatric Sle ◽  
History Of ◽  
Pediatric Onset

Systemic lupus erythematosus (SLE) is an autoantibody-related disease that affects multiple organs. Stercoral colitis (SC) is a rare type of inflammatory colitis with a high mortality rate. Here, we report the first case of pediatric-onset lupus in a case complicated by stercoral colitis. We also conducted a literature review of patients with SC under 30 years old to provide useful clues for rapid diagnosis at a young age. A 28-year-old female with a history of lupus and neuropsychiatric SLE was admitted with severe abdominal pain. She was found to have stercoral colitis during surgery. Two years later, the patient underwent Hartman's operation due to ischemia of the colon. In addition, 10 patients younger than 30 years old with a diagnosis of SC were analyzed based on clinical presentation, physical examination, laboratory exam, imaging and treatment. All cases had a favorable outcome without mortality. Stercoral colitis is a rare but lethal complication, emphasizing the importance of a multidisciplinary approach. Differential diagnosis should include stercoral colitis for patients with SLE developing unexplained sharp abdominal pain.

scholarly journals Complement deficiency in pediatric-onset systemic lupus erythematosus

2018 ◽  
Vol 10 (02) ◽  
pp. 232-236 ◽  
Author(s):  
Parisa Afzali ◽  
Anna Isaeian ◽  
Peyman Sadeghi ◽  
Bobak Moazzami ◽  
Nima Parvaneh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Musculoskeletal Symptoms ◽  
Complement C3 ◽  
Complement Deficiency ◽  
Control Group ◽  
Systemic Lupus ◽  
Cutaneous Manifestations ◽  
Pediatric Onset ◽  
Onset Systemic Lupus Erythematosus

Abstract BACKGROUND: Pediatric-onset systemic lupus erythematosus (pSLE) accounts for about 10%-20% of all patients with SLE. Deficiencies in early complement components of the classical pathway are the strong genetic risk factor for the development of SLE. In this study, clinical and laboratory manifestations of both complement-deficient and normal complement pSLE patients were compared. MATERIALS AND METHODS: To investigate clinical and immunological manifestations of pSLE in Iran, 36 consecutive pSLE patients (onset before 18 years) who were followed up over a period of 2 years, were studied. Complement C1q and C2 levels were measured using radial immunodifusion assay and complement C3 and C4 levels were measured using nephelometry. Medical records were retrospectively evaluated from patient database of Children Medical Center Hospital. Data were assessed through descriptive analysis (confidence interval = 95%), paired t-test, and Pearson correlation test. RESULTS: Twenty-one patients (58%) had at least one component of complement deficiency. Ten patients (27%) had low C1q level, 11 patients (30.5%) had low C2, nine patients (25%) had low C3, and four patients (11%) had low C4 level. Serum level of complement in pSLE was significantly lower than the control group, except C4 (P = 0.005). The low C1q patients had an earlier age of onset of disease (P < 0.0001). The cutaneous manifestations were more frequent and much more severe in pSLE with low complement (100% vs. 73%). The frequency of renal and musculoskeletal symptoms was equal, but renal morbidity was more common in pSLE with low complement. Positivity for anti-ds-DNA was less common in pSLE with low complement (71% vs. 86%). CONCLUSION: In pSLE patients with early disease onset and more aggressive SLE manifestations and negative anti-ds-DNA test, complement deficiency should be considered.

scholarly journals The Impact of Serum Anti-neutrophil Cytoplasmic Antibody on Clinical Characteristics and Outcomes in Pediatric-Onset Systemic Lupus Erythematosus Patients

2021 ◽  
Vol 8 ◽  
Author(s):  
Chun-Chun Gau ◽  
Min-Hua Tseng ◽  
Chao-Yi Wu ◽  
Huang-Yu Yang ◽  
Jing-Long Huang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Characteristics ◽  
Laboratory Data ◽  
Systemic Lupus ◽  
Retrospective Case ◽  
Clinical Presentations ◽  
The Impact ◽  
Pediatric Onset ◽  
Onset Systemic Lupus Erythematosus

Background: Systemic lupus erythematosus (SLE), an autoimmune disease, is characterized by the overproduction of autoantibodies. Anti-neutrophil cytoplasmic antibodies (ANCAs) have been recognized in SLE for decades. To date, their association with SLE disease activity, especially in pediatric-onset SLE (pSLE) patients, is limited.Methods: We conducted a retrospective case-control study of pSLE patients with ANCAs from 2010 to 2020. Clinical characteristics, laboratory data, renal histological features, treatment and outcomes were analyzed.Results: A total of 70 pediatric-onset SLE patients (9 ANCA-positive vs. 61 ANCA-negative) with a median age of 12.23 years (age ranging from 4 years to 18 years) at diagnosis were enrolled. Among patients with ANCAs, MPO-ANCA was found in seven and PR3-ANCA in two of those cases. Patients with ANCAs had a tendency to have hematuria compared with those without ANCAs (66 vs. 24.6%, respectively; p = 0.026). Of the 70 SLE patients, 8 with ANCAs and 44 without ANCAs underwent renal biopsies. Patients with ANCAs (25%, 2/8) were more likely to lack the typical full-house pattern in their renal immunofluorescence (IF) staining.Conclusion: pSLE patients with ANCAs tend to have hematuria and an absence of typical IF histology. However, patients with and without ANCAs showed no difference in their clinical presentations and treatment outcomes.

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