Search

Cryopyrin-associated periodic syndromes (CAPS) are a group of autoinflammatory diseases associated with NLRP3 gain of function mutations. CAPS associated mutations are found predominantly in exon 3. The objective of this study is to describe a new variant on NRLP3 gene and its phenotype. Case report description of a new NRLP3 pathogenic variant and literature case-based search through INFEVERS database. A 21-year old male who presented multiple tonic–clonic seizures on his 3rd day of life. At age 2, he had recurrent central facial palsy, high fever (40 °C), painful and persistent oral ulcers, abdominal pain, nausea and vomiting, and delayed neuropsychomotor development, with polyarthritis in wrists and knees. Over the years, several symptoms were observed: livedo reticularis, Raynaud’s phenomenon, positive pathergy test, heat allodynia, extremely painful genital ulcers, and sporadic conjunctivitis. Laboratory studies revealed persistently elevated inflammatory markers and serum amyloid protein A (30 μg/l). The genetic panel for autoinflammatory diseases revealed heterozygous mutation in the NLRP3, (c.2068G > C, p.E690Q) with 0% of frequency in the general population. The patient denies rash and did not have frontal bossing or patellar overgrowth. We found a positive familial history on mother and brother, who carried the same mutation. The patient was started on canakinumab which controlled his symptoms. Currently, 241 missense variants in the NLRP3 have been described. We presented a new mutation in exon 3 of the NRLP3 gene in a patient that fulfills clinical criteria for CAPS who had complete clinical response to Canakinumab, supporting the idea that this mutation is pathogenic.

Background Fabry disease (FD) is a rare, X-linked, multisystemic lysosomal storage disorder (LSD) that results from a deficiency in the hydrolase alpha-galactosidase A (⍺-GalA). During childhood, classic FD symptomatology is rare. The majority of children may show non-specific symptoms, including in the musculoskeletal system. The prevalence of FD among juvenile idiopathic arthritis (JIA) patients is unknown. Objective This study aimed to identify the frequency of FD in a JIA cohort, characterizing early clinical symptoms, enzyme titers, and GLA genotyping. Methods Children with JIA followed in a tertiary Children Hospital cohort were selected. Clinical, laboratory and familiar information were recorded. Molecular genetic testing to detect GLA gene mutations was performed in girls and enzymatic analysis in boys. Results In 89 patients (56.2% female, age at disease onset: 8.93 ± 4.35 years), one male (1.12%) patient presented pathogenic mutation in GLA gene, c.1244 T > C p.L415P, one female patient had a variant of uncertain significance c.38C > T (p.Ala13Val). Three additional (3.4%) patients had the enzymatic activity of alpha-galactosidase slightly decreased. We observed the presence of intronic variants in 44.44% of patients in our cohort: c.1000-22C > T; c.370-81_-77del; c.640-16A > G; c.10C > T; c.548-125C > G and c.-12G > A. These variants and their combination were associated with clinical symptoms in our cohort. Conclusions The incidence of FD in our cohort was 1.12%. Intronic variants were associated with symptoms previously described in the literature. Screening for FD in JIA may be a reasonable strategy for those with an atypical pattern of pain.

Cryopyrin-associated periodic syndromes (CAPS) are a group of autoinflammatory diseases associated with NLRP3 gain of function mutations. CAPS associated mutations are found predominantly in exon 3. The objective of this study is to describe a new variant on NRLP3 gene and its phenotype. Case report description of a new NRLP3 pathogenic variant and literature case-based search through INFEVERS database. A 21-year old male who presented multiple tonic-clonic seizures on his 3rd day of life. At age 2, he had recurrent central facial palsy, high fever (40 °C), painful and persistent oral ulcers, abdominal pain, nausea and vomiting, and delayed neuropsychomotor development, with polyarthritis in wrists and knees. Over the years, several symptoms were observed: livedo reticularis, Raynaud's phenomenon, positive pathergy test, heat allodynia, extremely painful genital ulcers, and sporadic conjunctivitis. Laboratory studies revealed persistently elevated inflammatory markers and serum amyloid protein A (30 μg/l). The genetic panel for autoinflammatory diseases revealed heterozygous mutation in the NLRP3, (c.2068G C, p.E690Q) with 0% of frequency in the general population. The patient denies rash and did not have frontal bossing or patellar overgrowth. We found a positive familial history on mother and brother, who carried the same mutation. The patient was started on canakinumab which controlled his symptoms. Currently, 241 missense variants in the NLRP3 have been described. We presented a new mutation in exon 3 of the NRLP3 gene in a patient that fulfills clinical criteria for CAPS who had complete clinical response to Canakinumab, supporting the idea that this mutation is pathogenic.

Made available in DSpace on 2020-12-10T20:01:32Z (GMT). No. of bitstreams: 0 Previous issue date: 2020-06-05 Objective This study aimed to assess the safety and immunogenicity of the quadrivalent human papillomavirus (qHPV) vaccination in childhood-onset systemic lupus erythematosus (cSLE) patients. Methods Volunteer cSLE patients aged 9-20 years and healthy controls (HC) were enrolled to receive a two- or three-dose qHPV vaccination schedule from March 2014 to March 2016. Study visits were performed before the first dose, one month after the second and third doses and one year after the first dose. In each study visit, disease activity and adverse events following vaccination were analyzed, and a serum sample was collected for testing antibody concentrations. Participant recruitment was conducted in 15 Brazilian paediatric rheumatology units. Of the 256 cSLE patients included, 210 completed the two- or three-dose schedules; 15 had previously received one dose, and 18 had received two doses of the vaccine. The analysis was based on intention-to-treat so that participants who did not complete the entire study protocol were also included. Results No severe adverse events were related to the vaccination. Disease activity was generally low and remained stable or even improved. The HC presented 100% seropositivity to HPV16 and HPV18, whereas the two- and three-dose cSLE groups presented 93% and 83% versus 97% and 91%, respectively. One year after the first dose, seropositivity of the three-dose cSLE group was 91% to HPV16 and 84% to HPV18. Conclusions HPV vaccination in cSLE patients is safe and immunogenic. Since the seropositivity to HPV16 and HPV18 was higher for the three-dose schedule group, this regimen should be recommended for cSLE patients. Univ Med Ctr Utrecht, Wilhelmina Childrens Hosp, Dept Paediat Immunol & Rheumatol, Utrecht, Netherlands Univ Fed Parana, Hosp Clin, Dept Paediat, Curitiba, Parana, Brazil Univ Sao Paulo, Dept Paediat, Fac Med Ribeirao Preto, Sao Paulo, Brazil Univ Estado Rio De Janeiro, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Univ Sao Paulo, Hosp Clin, Inst Crianca, Dept Paediat Rheumatol,Fac Med, Sao Paulo, Brazil Hosp Infantil Albert Sabin, Dept Paediat Rheumatol, Fortaleza, Ceara, Brazil Univ Florida, Coll Med, Dept Paediat Immunol & Rheumatol, Gainesville, FL USA Univ Estadual Campinas, Dept Paediat Rheumatol, Sao Paulo, Brazil Hosp Crianca Brasilia Jose Alencar, Dept Paediat Rheumatol, Brasilia, DF, Brazil Univ Estadual Paulista, Dept Paediat Rheumatol, Botucatu, SP, Brazil Univ Fed Rio de Janeiro, Inst Puericultura & Pediat Martagao Gesteira IPPM, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Univ Fed Rio de Janeiro, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Hosp Infantil Darcy Vargas, Dept Paediat Rheumatol, Sao Paulo, Brazil Hosp Estadual Infantil Nossa Senhora da Gloria, Dept Paediat Rheumatol, Vitoria, ES, Brazil Santa Casa Sao Paulo, Dept Paediat Rheumatol, Sao Paulo, Brazil Univ Fed Bahia, Dept Paediat Rheumatol, Fac Med, Salvador, BA, Brazil Univ Fed Sao Paulo, Dept Paediat Rheumatol, Sao Paulo, Brazil Hosp Pequeno Principe, Dept Paediat Rheumatol, Curitiba, Parana, Brazil Natl Inst Publ Hlth & Environm RIVM, Ctr Infect Dis Control, Bilthoven, Netherlands Fac Med Barretos FACISB, Dept Paediat Rheumatol, Barretos, Brazil Univ Estadual Paulista, Dept Paediat Rheumatol, Botucatu, SP, Brazil

Background To our knowledge the influence of ethnic background in childhood-onset SLE (cSLE) presentation was not evaluated in a large population of Latin American country. Objectives To assess demographic data, clinical manifestations, laboratory abnormalities and disease activity score in cSLE patients according to ethnic groups at diagnosis Methods This multicenter study included cSLE patients(ACR criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents’ and all four grandparents’ self-reported ethnicity. The statistical analysis was performed using the Bonferroni’s correction(p Results According to ethnic groups, 1,537 cSLE patients were classified in: Caucasian (n=786), African-Latin American(n=526), Asian(n=8) and others/unknown(n=217). Comparisons between 1,312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis[12.2(2.6-18) vs. 12.1(0.3-18) years,p=0.234], time interval to diagnosis[0.25(0-12) vs. 0.3(0-10) years,p=0.034] and SLEDAI-2K score[14(0-55) vs. 14(0-63),p=0.781] in both groups. The mean number of diagnostic criteria according to SLICC(6.47±1.911 vs. 5.81±1.631, p Conclusion Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable to Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients. Disclosure of Interests None declared

To describe the prevalence and risk factors associated with corneal vortex keratopathy in a childhood-onset systemic lupus erythematosus (c-SLE) cohort. Consecutive outpatients with c-SLE were evaluated by a pediatric ophthalmologist and pediatric rheumatologist in an outpatient clinic setting in an urban Children’s Hospital. Demographic, clinical, laboratory, and disease characteristics were documented for each patient. Cumulative drug dosage, Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), and Systemic Lupus Erythematosus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR-DI) scores were calculated. A total of 76 c-SLE patients (61 (80.26%) females; mean age = 17.9 (SD ± 3.07)) were included. Ophthalmologic abnormalities were observed in 36 (47.36%) patients of which 16 (21.10%) had corneal vortex keratopathy (p = 0.03). c-SLE patients with corneal vortex keratopathy were all female. We did not observe any additional clinical, laboratory, or treatment feature associated with corneal vortex keratopathy. We observed a high prevalence of corneal vortex keratopathy in female c-SLE. We hypothesize that this finding may be an initial, dose-related toxicity due to antimalarial use. Follow-up studies are necessary to determine if these changes are an early predictor of retinal toxicity due to antimalarial in c-SLE. • Corneal vortex keratopathy was frequently observed in female patients with c-SLE on a chloroquine medication. • Corneal vortex keratopathy may be an early marker of chloroquine retinopathy.

Made available in DSpace on 2019-10-06T16:49:50Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-01-01 Objective: To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents’ and all four grandparents’ self-reported ethnicity. The statistical analysis was performed using the Bonferroni’s correction (p < 0.0027). Results: According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6–18) vs. 12.1(0.3–18) years, p = 0.234], time interval to diagnosis [0.25(0–12) vs. 0.3(0–10) years, p = 0.034], and SLEDAI-2K score [14(0–55) vs. 14(0–63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 ± 1.911 vs. 5.81 ± 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. Conclusions: Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.Key Points• Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian.• Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of African-Latin American cSLE patients.• African-Latin American cSLE patients had more often anti-phospholipid antibodies and hypocomplementemia.• The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients. Pediatric Rheumatology Unit Children’s Institute Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César Pediatric Rheumatology Unit Hospital Jose Alencar Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Unit Universidade Federal de Sao Paulo Pediatric Rheumatology Division Sao Paulo State University (UNESP) Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit Ribeirao Preto Medical School – University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit Rio de Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Pedro Ernesto University Hospital Pediatric Rheumatology Unit Albert Sabin Children’s Hospital Pediatric Rheumatology Unit Hospital Darcy Vargas Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo Rheumatology Division - Universidade Federal do Rio de Janeiro Hospital Universitário Clementino Fraga Filho Pediatric Rheumatology Unit Lauro Vanderley University Hospital Pediatric Rheumatology Unit Federal University of Pará Pediatric Rheumatology Unit Obras Sociais Irmã Dulce Pediatric Rheumatology Unit Hospital Evangélico de Curitiba Pediatric Rheumatology Unit Hospital Criança Conceição Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Federal University of Minas Gerais Pediatric Rheumatology Unit Pontifícia Catholic University of Sorocaba Pediatric Rheumatology Division Sao Paulo State University (UNESP)

Made available in DSpace on 2018-12-11T17:24:18Z (GMT). No. of bitstreams: 0 Previous issue date: 2018-09-01 Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) Objective: The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods: A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions: Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition. Pediatric Rheumatology Unit Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Unit Universidade Federal de Sao Paulo Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Division Sao Paulo State University (UNESP) Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit Rio de Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Hospital Jose Alencar Pediatric Rheumatology Unit Pedro Ernesto University Hospital Pediatric Rheumatology Unit Albert Sabin Children’s Hospital Pediatric Rheumatology Unit Hospital Darcy Vargas Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo Rheumatology Division Hospital Universitário Clementino Fraga Filho Pediatric Rheumatology Unit Lauro Vanderley University Hospital Pediatric Rheumatology Unit Federal University of Pará Pediatric Rheumatology Unit Obras Sociais Irmã Dulce Pediatric Rheumatology Unit Hospital Evangélico de Curitiba Pediatric Rheumatology Unit Hospital Criança Conceição Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Federal University of Minas Gerais Pediatric Rheumatology Unit Hospital Municipal Piedade Pediatric Rheumatology Division Sao Paulo State University (UNESP) FAPESP: 03756-4

Blau’s syndrome is an autosomal-dominant, autoinflammatory disease characterized by a non caseous granulomatous inflammation,presenting with arthritis, dermatitis and uveitis, caused by mutations of the CARD15/NOD2. Patients are treated with high doses of oral corticosteroids,and if the therapeutic response is unsatisfactory,additional treatment with immunosuppressive agents is the best choice,such as: Methotrexate,Cyclosporine,Anti-TNF and Canakinumab (Anti-IL1).

OBJETIVOS: Identificar fatores preditivos iniciais de envolvimento renal nas crianças e adolescentes com púrpura de Henoch-Schönlein. MÉTODOS: Por um período de 21 anos, os prontuários de 142 pacientes com diagnóstico de púrpura de Henoch-Schönlein admitidos em nosso Hospital Universitário foram revistos. Os fatores preditivos iniciais avaliados nos primeiros 3 meses incluíram: dados demográficos, manifestações clínicas (púrpura palpável persistente, artrite, dor abdominal, dor abdominal intensa, sangramento gastrointestinal, orquite, envolvimento do sistema nervoso central e hemorragia pulmonar), exames laboratoriais (níveis séricos de IgA) e tratamento utilizado (corticosteróides, imunoglobulina endovenosa e medicação imunossupressora). Os pacientes foram divididos em dois grupos (com presença ou ausência de nefrite) e avaliados de acordo com a análise univariada e multivariada. RESULTADOS: Nefrite foi evidenciada em 70 pacientes (49,3%). A análise univariada revelou que dor abdominal intensa (p = 0,0049; OR = 1,6; IC95% 1,18-2,21), sangramento gastrointestinal (p = 0,004; OR = 1,6; IC95% 1,10-2,26) e uso dos corticosteróides (p = 0,0012; OR = 1,7; IC95% 1,28-2,40) foram associados com uma maior incidência de envolvimento renal. Na análise multivariada, a regressão logística mostrou que a única variável independente na predição da ocorrência de nefrite foi dor abdominal intensa (p < 0,012; OR = 2,593; IC95% 1,234-5,452). CONCLUSÕES: Dor abdominal intensa representou um preditor significativo da nefrite na púrpura de Henoch-Schönlein. Conseqüentemente, os pacientes pediátricos com esta manifestação clínica devem ser rigorosamente seguidos, devido ao maior risco de acometimento renal. OBJECTIVES: To identify initial predictive factors of renal involvement in children and adolescents with Henoch-Schönlein purpura. METHODS: We reviewed the medical records of 142 patients admitted to our University Hospital over a 21-year period with a diagnosis of Henoch-Schönlein purpura. The initial predictive factors assessed, observed during the first 3 months, included: demographic data, clinical manifestations (persistent palpable purpura, arthritis, abdominal pain, severe abdominal pain, gastrointestinal bleeding, orchitis, central nervous system involvement and pulmonary hemorrhage), laboratory tests (serum IgA levels) and treatment given (corticosteroids, intravenous immunoglobulin and immunosuppressive drugs). Patients were divided into two groups (presence or absence of nephritis) and assessed by univariate and multivariate analysis. RESULTS: Evidence of nephritis was detected in 70 patients (49.3%). The univariate analysis revealed that severe abdominal pain (p = 0.0049; OR = 1.6; 95%CI 1.18-2.21), gastrointestinal bleeding (p = 0.004; OR = 1.6; 95%CI 1.10-2.26) and corticosteroid use (p = 0.0012; OR = 1.7; 95%CI 1.28-2.40) were all associated with increased incidence of renal involvement. In the multivariate analysis, logistic regression demonstrated that the only independent variable that predicted nephritis was intense abdominal pain (p < 0.012; OR = 2.593; 95%CI 1.234-5.452). CONCLUSIONS: Severe abdominal pain was a significant predictor of nephritis in Henoch-Schönlein purpura. Consequently, pediatric patients exhibiting this clinical manifestation should be rigorously monitored, due to the increased risk of renal involvement.

OBJETIVOS: Identificar fatores preditivos iniciais de envolvimento renal nas crianças e adolescentes com púrpura de Henoch-Schönlein. MÉTODOS: Por um período de 21 anos, os prontuários de 142 pacientes com diagnóstico de púrpura de Henoch-Schönlein admitidos em nosso Hospital Universitário foram revistos. Os fatores preditivos iniciais avaliados nos primeiros 3 meses incluíram: dados demográficos, manifestações clínicas (púrpura palpável persistente, artrite, dor abdominal, dor abdominal intensa, sangramento gastrointestinal, orquite, envolvimento do sistema nervoso central e hemorragia pulmonar), exames laboratoriais (níveis séricos de IgA) e tratamento utilizado (corticosteróides, imunoglobulina endovenosa e medicação imunossupressora). Os pacientes foram divididos em dois grupos (com presença ou ausência de nefrite) e avaliados de acordo com a análise univariada e multivariada. RESULTADOS: Nefrite foi evidenciada em 70 pacientes (49,3%). A análise univariada revelou que dor abdominal intensa (p = 0,0049; OR = 1,6; IC95% 1,18-2,21), sangramento gastrointestinal (p = 0,004; OR = 1,6; IC95% 1,10-2,26) e uso dos corticosteróides (p = 0,0012; OR = 1,7; IC95% 1,28-2,40) foram associados com uma maior incidência de envolvimento renal. Na análise multivariada, a regressão logística mostrou que a única variável independente na predição da ocorrência de nefrite foi dor abdominal intensa (p < 0,012; OR = 2,593; IC95% 1,234-5,452). CONCLUSÕES: Dor abdominal intensa representou um preditor significativo da nefrite na púrpura de Henoch-Schönlein. Conseqüentemente, os pacientes pediátricos com esta manifestação clínica devem ser rigorosamente seguidos, devido ao maior risco de acometimento renal.

Systemic juvenile idiopathic arthritis (SJIA) is characterized by fevers, rash, and chronic arthritis, and interleukin -1 (IL-1) and IL-6 inhibitors seems to be effective treatments. Pulmonary arterial hypertension (PAH) and macrophage activation syndrome (MAS), which is a unremitting fever, coagulopathy, pancytopenia, and multiple organ dysfunction. These complications can be fatal and may be the result of severe uncontrolled systemic disease activity or influenced by medication exposure.

The visceral leishmaniosis (VL), or Calazar, is a chronic severe systemic disease, potentially fatal to humans. Currently, VL is the prototype of a specific immune dysfunction resulting from parasitism of leishmania donovani in macrophages, producing a broad spectrum of clinical and immunological reversible only with specific treatment. Serum Analysis from infected adult patients demonstrated the presence of autoantibodies against cellular and humoral components, and circulating immune complexes.

The Polymyositis (PM) is an inflammatory myopathy with symmetrical proximal muscle weakness, especially in pelvic, shoulder girdle and cervical musculature. It may occur primary or secondary to other diseases. Diagnosis is made by exclusion of other etiologies, such as infections, endocrinopathies, metabolics diseases and Bohan and Peter criteria. Leptospirosis is a zoonosis of worldwide distribution, that can be asymptomatic, with clinical presentation ranging from an acute febrile illness, with headache, severe myalgia, fever, arthralgia, to a severe syndrome of multiorgan dysfunction (Weill syndrome) and the diagnosis may be missed unless the physician has a high index of suspicion for the disease.