MARIANA PAES LEME FERRIANI
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- Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus(2015) FERRIANI, Mariana Paes Leme; SILVA, Marco Felipe Castro; PEREIRA, Rosa Maria Rodrigues; TERRERI, Maria Teresa; MAGALHAES, Claudia Saad; BONFA, Eloisa; PASTORINO, Antonio C.; SANTOS, Maria Carolina dos; APPENZELLER, Simone; FERRIANI, Virginia Paes Leme; LEN, Claudio Arnaldo; SALLUM, Adriana Maluf Elias; LIBORIO, Jonatas; CASTRO, Tania Caroline Monteiro de; SILVA, Clovis ArturBackground: 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. (C) 2015 S. Karger AG, Basel
- A Multicenter Study of Invasive Fungal Infections in Patients with Childhood-onset Systemic Lupus Erythematosus(2015) SILVA, Marco F.; FERRIANI, Mariana P.; TERRERI, Maria T.; PEREIRA, Rosa M.; MAGALHAES, Claudia S.; BONFA, Eloisa; CAMPOS, Lucia M.; OKUDA, Eunice M.; APPENZELLER, Simone; FERRIANI, Virginia P.; BARBOSA, Cassia M.; RAMOS, Valeria C.; LOTUFO, Simone; SILVA, Clovis A.Objective. To study the prevalence, risk factors, and mortality of invasive fungal infections (IFI) in patients with childhood-onset systemic lupus erythematosus (cSLE). Methods. A retrospective multicenter cohort study was performed in 852 patients with cSLE from 10 pediatric rheumatology services. An investigator meeting was held and all participants received database training. IFI were diagnosed according to the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group Consensus Group criteria (proven, probable, and possible). Also evaluated were demographic, clinical, and laboratory data, and disease activity [SLE Disease Activity Index 2000 (SLEDAI-2K)], cumulative damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), treatment, and outcomes. Results. IFI were observed in 33/852 patients (3.9%) with cSLE. Proven IFI was diagnosed in 22 patients with cSLE, probable IFI in 5, and possible IFI in 6. Types of IFI were candidiasis (20), aspergillosis (9), cryptococcosis (2), and 1 each disseminated histoplasmosis and paracoccidioidomycosis. The median of disease duration was lower (1.0 vs 4.7 yrs, p < 0.0001) with a higher current SLEDAI-2K [19.5 (0-44) vs 2 (0-45), p < 0.0001] and current prednisone (PRED) dose [50 (10-60) vs 10 (2-90) mg/day, p < 0.0001] in patients with IFI compared with those without IFI. The frequency of death was higher in the former group (51% vs 6%, p < 0.0001). Logistic regression analysis revealed that SLEDAI-2K (OR 1.108, 95% CI 1.057-1.163, p < 0.0001), current PRED dose (OR 1.046, 95% CI 1.021-1.071, p < 0.0001), and disease duration (OR 0.984, 95% CI 0.969-0.998, p = 0.030) were independent risk factors for IFI (R-2 Nagelkerke 0.425). Conclusion. To our knowledge, this is the first study to characterize IFI in patients with cSLE. We identified that disease activity and current glucocorticoid use were the main risk factors for these life-threatening infections, mainly in the first years of disease course, with a high rate of fatal outcome.
- Characterization of chronic arthritis in a multicenter study of 852 childhood-onset systemic lupus erythematosus patients(2016) SAKAMOTO, Ana Paula; SILVA, Clovis Artur; FERRIANI, Mariana Paes Leme; PEREIRA, Rosa Maria Rodrigues; BONFA, Eloisa; SAAD-MAGALHAES, Claudia; OKUDA, Eunice; APPENZELLER, Simone; GOMES, Francisco Hugo; CUNHA, Ana Luiza Garcia; SALUME, Mirna Henriques Tomich; PIOTTO, Daniela Petry; TERRERI, Maria TeresaChronic arthritis (CA) is an unusual condition in childhood-onset systemic lupus erythematosus (cSLE) and data in children is very limited. The aim of the study is to assess CA in a large population of cSLE patients, in a multicenter cross-sectional study including 852 cSLE patients followed in ten Pediatric Rheumatology referral services in state of So Paulo, Brazil. CA was observed in 32/852 (3.7 %) cSLE patients mostly in hands and ankles. Chronic monoarthritis was diagnosed in four cSLE patients, oligoarthritis in nine and polyarthritis in 19. In the latter group, six had rhupus syndrome. Two oligoarticular patients had Jaccoud's arthropathy. CA was an isolated manifestation observed at disease onset in 13/32 (41 %) cSLE patients, and juvenile idiopathic arthritis (JIA) was the first diagnosis in 18/32 (56 %). The comparison of last visit of patients with CA and without this manifestation revealed higher frequency of splenomegaly (28 vs. 11 %, p = 0.002). The median of SLICC/ACR-DI score [1(0-9) vs. 0(0-7), p = 0.003] was significantly higher in CA patients compared to patients without this manifestation, likewise the frequency of musculoskeletal damage (31 vs. 9 % p = 0.001). Frequencies of treatment with nonsteroidal anti-inflammatory drugs (75 vs. 26 %, p < 0.0001), hydroxychloroquine sulfate (87 vs. 59 %, p = 0.001) and methotrexate (47 vs. 22 %, p = 0.001) were significantly higher in CA patients. This large multicenter study allowed us to characterize CA as a rare and early manifestation of cSLE, frequently mimicking JIA at disease onset. It is predominantly polyarticular, involving more often hands and ankles and it is associated with significant musculoskeletal accrual damage.
- Evans Syndrome at Childhood-Onset Systemic Lupus Erythematosus Diagnosis: A Large Multicenter Study(2016) LUBE, Gabriella E.; FERRIANI, Mariana Paes Leme; CAMPOS, Lucia Maria Arruda; TERRERI, Maria Teresa; BONFA, Eloisa; MAGALHAES, Claudia Saad; AIKAWA, Nadia Emi; PIOTTO, Daniela Petry; PERACCHI, Octavio Augusto Bedin; SANTOS, Maria Carolina dos; APPENZELLER, Simone; FERRIANI, Virginia Paes Leme; PEREIRA, Rosa Maria Rodrigues; SILVA, Clovis ArturBackground. Evans syndrome (ES) in childhood-onset systemic lupus erythematosus (cSLE) patients has been rarely reported and limited to small populations. Procedures. A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services including 850 patients with cSLE. ES was assessed at disease diagnosis and defined by the combination of immune thrombocytopenia and autoimmune hemolytic anemia. Results. ES was observed in 11 of 850 (1.3%) cSLE patients. The majority of them had hemorrhagic manifestations (91%) and active disease (82%). All patients with ES were hospitalized and none died. Comparisons of cSLE patients with and without ES at diagnosis revealed similar frequencies of female gender, multiorgan involvement, autoantibodies profile, and low complement (P > 0.05). Patients with ES had a lower frequency of malar rash (9% vs. 53%, P = 0.003) and musculoskeletal involvement (18% vs. 69%, P = 0.001) than those without this complication. The frequencies of intravenous methylprednisolone (82% vs. 43%, P = 0.013) and intravenous immunoglobulin use (64% vs. 3%, P < 0.0001) were significantly higher in the ES group, with similar current prednisone dose between groups (1.1 [0.76-1.5] vs. 1.0 mg/kg/day [0-30], P = 0.195). Conclusions. Our large multicenter study identified ES as a rare and severe initial manifestation of active cSLE with good outcome. Diagnosis is challenging due to the lack of typical signs and symptoms of lupus and the requirement to exclude infection and primary immunodeficiency. (C) 2016 Wiley Periodicals, Inc.