Evolución de una vasculitis renal asociada a anticuerpos anti-citoplasma de neutrófilos (ANCA) durante un embarazo: caso clínico / Antineutrophil cytoplasmic antibody (ANCA) associated renal vasculitis and pregnancy: report of one case

ANCA mediated vasculitis mainly occur between the fourth and fifth decade of life; therefore, it is very uncommon to see pregnant patients with the disease. Vasculitis may affect significantly the course of pregnancy; in turn pregnancy can change the course of vasculitis. We report a 20 years old woman with ANCA-mediated renal vasculitis lasting 10 years who consulted with a pregnancy of 15 weeks. She was in remission and had amenorrhea attributed to ovarian toxicity due to cyclophosphamide. Pregnancy had an uneventful course with spontaneous delivery at the 37th week, giving birth to a healthy newborn. Proteinuria increased during the course of pregnancy with a mild deterioration of kidney function. During the year after delivery, she had nephrotic proteinuria and a worsening of renal function.

Pro-calcitonin and inflammation in chronic hemodialysis / Pro-calcitonina e inflamación en hemodiálisis crónica

Procalcitonin (PCT) has emerged as a marker of infection, a frequent complication in hemodialysis (HD). We analyzed PCT levels in chronic non-acutely infected HD subjects, assessed its correlation with inflammatory and nutritional markers and propose a PCT reference value for non-infected HD patients.In an observational cross-sectional study, 48 chronic HD patients and 36 controls were analyzed. Variables: age, gender, time on HD; diabetes; vascular access, PCT, C-reactive protein (CRP), albumin, malnutrition inflammatory score (MIS), hematocrit, leukocyte count, and body mass index (BMI). Subsequently, control (G1, n = 36, 43%) vs. non-infected patients (G2, n = 48, 57%) groups were compared. In control subjects (G1), age: 54.3 ± 13.7 years, range (r): 30-81; males: 19 (53%); median PCT 0.034 ng/ml (r: 0.02-0.08); median CRP 0.80 mg/dl (r: 0.36-3.9); p95 PCT level: 0.063 ng/ml. In G2, age: 60.2 ± 15.2 years; males 32 (67%), time on HD: 27.0 ± 24.4; diabetics: 19 (32%); median PCT: 0.26 ng/ml (r: 0.09-0.82); CRP: 1.1 mg/dl (r: 0.5-6.2); p95 PCT level: 0.8 ng/ml. In control subjects, PCT and CRP were significantly lower than in G2: PCT: 0.034 vs. 0.26 ng/ml, p = 0.0001; CRP: 0.8 vs. 1.1 mg/dl, p = 0.0004. PCT-CRP correlation in G2: ρ = 0.287, p = 0.048. PCT and CRP concentrations are elevated in chronic non-acutely infected HD subjects, independently of infection, diabetes and vascular access. A p95 PCT level of 0.8 ng/ml may be considered as the upper normal reference value in non-acutely infected HD subjects. The PCT cut-off level in HD is yet to be determined in HD.

La procalcitonina (PCT) puede ser un marcador de infección en la hemodiálisis (HD). Analizamos los niveles de PCT en sujetos sin infección aguda en HD crónica, su correlación con marcadores inflamatorios y nutricionales y, de acuerdo a ello, proponemos niveles de referencia de PCT. En un estudio observacional transversal se estudiaron 48 pacientes en HD y 36 controles. Variables: edad; sexo, tiempo en HD; diabetes; acceso vascular, PCT, proteína C-reactiva (PCR), albúmina, score de malnutrición-inflamación, hematocrito, recuento leucocitario, e índice de masa muscular (IMC). En los controles se determinaron PCT y PCR. Se comparó grupo control (G1, n = 36, 43%) vs. pacientes (G2, n = 48, 57%). G1: edad, 54.3 ± 13.7, rango (r): 30-81 años; hombres: 19 (53%); PCT mediana: 0.034 ng/ml (r: 0.020-0.080); PCR mediana: 0.8 mg/dl (r: 0.36-3.9); el nivel p95 de PCT: 0.063 ng/ml. En el G2, edad media 60.2 ± 15.2 años, hombres: 32 (66%), tiempo en HD: 27.0 2 4.4; diabéticos: 19 (32%); PCT: 0.26 ng/ml (r: 0.09-0.82); PCR: 1.1 mg/dl (r: 0.5-6.2); p95 PCT: 0.8 ng/ml. En G1 los niveles de PCT y PCR fueron significativamente más bajos que en G2: PCT: 0.034 vs. 0.26 ng/ml, p = 0.0001; PCR: 0.8 vs 1.1 mg/dl, p = 0.0004. Correlación PCT- PCR en G2: ρ = 0.287, p = 0.048. La PCT y la PCR están elevadas en HD crónica independientemente de infección, diabetes y acceso vascular. Se propone p95 de PCT de 0.8 ng/ml como límite superior del intervalo de referencia en sujetos sin infección aguda en HD. El valor de PCT en HD está por determinarse.

Evaluation of D-dimer serum levels among patients with chronic urticaria, psoriasis and urticarial vasculitis / Avaliação dos níveis séricos de D-dímeros entre doentes com urticária crônica, psoríase e urticária vasculite

BACKGROUND: It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. OBJECTIVE: To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. PATIENTS AND METHODS: The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. RESULTS: Patients with active chronic urticaria had the highest serum levels of D-dimer (p<0.01), when compared to patients with CU under remission and the control group (patients with psoriasis). CONCLUSIONS: Patients with active chronic urticaria have higher serum levels of D-dimer, when compared to patients with chronic urticaria under remission and patients with psoriasis. We found elevated serum levels of D-dimer among patients with urticarial vasculitis. .

FUNDAMENTOS: Tem sido demonstrado que os neutrófilos, eosinófilos e monócitos, sob estímulo apropriado, podem expressar fator tecidual e, portanto, ativar a via extrínseca da coagulação. Realizamos um estudo transversal e caso-controle de pacientes com urticária crônica e pacientes com psoríase em nosso ambulatório para avaliar a produção de dímero-D. OBJETIVO: Avaliar níveis de dímero-D em pacientes com urticária crônica e sua possível correlação com a atividade da doença. PACIENTES E MÉTODOS: O estudo foi conduzido de outubro de 2010 até março de 2011. Nós selecionamos 37 pacientes consecutivos da Unidade de Alergia e Unidade de Psoríase, divididos em três grupos para análise estatística: (i) 12 pacientes com urticária crônica ativa; (ii) 10 pacientes com urticária crônica em remissão e (iii) 15 pacientes com psoríase (uma doença com a pele infiltrado inflamatório constituído por neutrófilos, linfócitos e monócitos). Outros cinco pacientes com vasculite urticariforme foram alocados em nosso estudo, mas não incluídos na análise estatística. Os níveis séricos de D-dímero foram medidos por Enzyme Linked Fluorescent Assay (ELFA), e os resultados foram medidos em ng / ml FEU. RESULTADOS: Os pacientes com urticária crônica ativa tinham níveis séricos mais altos de dímero-D (p <0,01), quando comparados aos pacientes com urticária crônica em remissão e ao grupo controle (pacientes com psoríase). CONCLUSÕES: Os pacientes com urticária crônica ativa têm níveis séricos mais elevados de dímero-D, quando comparados aos pacientes ...

Marcadores de inflamación endotelial subclínica en una familia con hiperaldosteronismo familiar tipo I por mutación de novo / Subclinical endothelial inflammation markers in a family with type I familial hyperaldosteronism caused by a de novo mutation

Background: Type I familial hyperaldosteronism is caused by the presence of a chimaetic gene CYPl 1B1/CYP11BZ which encodes an enzyme with aldosterone synthetase activityregulated by adrenocorticotrophic hormone (ACTH). Therefore, in patients with FH I is possible to normalize the aldosterone levels with glucocorticoid treatment. Recently it has been shown that aldosterone plays a role in the production of endothelial oxidative stress and subclinical inflammation. Aim: To evaluate subclinical endothelial inflammation markers, Me Metalloproteinase 9 (MMP-9) and ultrasensitive C reactive protein (usPCR), before and after glucocorticoid treatment in family members with FH-I caused by a de novo mutation. Patients and methods: We report three subjects with FH-I in a single family (proband, father and sister). We confirmed the presence of a chimaeric CYPl 1B1/CYP11B2 gene by ¡ong-PCR in all of them. Paternal grandparents were unaffected by the mutation. The proband was a 13year-old boy with hypertension stage 2 (in agree to The JointNational Committee VII, JNC-vIl), with an aldosterone/plasma rennin activity ratio equal to 161. A DNA paternity test confirmed the parental relationship between the grandparents and father with the index case. MMP-9 and usPCR levels were determined by gelatin zymography and nephelometry, respectively. Results: All affected subjects had approximately a 50 percent increase in MMP-9 levels. Only the father had an elevated usPCR. The endothelial inflammation markers returned to normal range after glucocorticoid treatment. Conclusions: We report a family canying a FH-I caused by a de novo mutation. The elevation of endothelial inflammation markers in these patients and its normalization after glucocorticoid treatment provides new insight about the possible deleterious effect of aldosterone on the endothelium.

Vasculitis ANCA-positivo en pacientes hipertiroideos usuarios de propiltiouracilo: Report of four cases / Antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis associated to the use of propylthiouracil

Antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis is an uncommon complication of the use of propylthiouracil. When it occurs, it affects multiple organs as any systemic vasculitis. We report three females and one male, aged 30, 40, 43 and 41 years respectively, that after a lapse of 12 to 28 months of propylthiouracil use, presented clinical signs of vasculitis. All had high titers of ANCA against myeloperoxidase. In three patients, a skin biopsy confirmed the diagnosis. The condition subsided when propylthiouracil was discontinued, but one female patient required the use of prednisone.

Raised platelet thiobarbituric acid-reacting substances in proliferative Eales' disease.

BACKGROUND: Platelets are an elective site for oxidative stress owing to their high content of polyunsaturated fatty acid. Increased lipid peroxidation and elevated platelet thiobarbituric acid-reacting substances (TBARS) signal oxidative stress. This possibly leads to retinal neovascularization in Eales' disease. METHODS: TBARS levels were estimated in consecutive cases of Eales' disease with neovascularisation (n = 26), Eales' disease without neovascularisation (n = 17) and healthy controls (n = 17). RESULTS: Platelet TBARS levels in the cases of Eales' disease with neovascularisation, Eales' disease without neovascularisation, and healthy controls were 0.66 +/- 0.1, 0.57 +/- 0.11 and 0.42 +/- 0.14 n moles TBARS formed/hour/10(8) platelets respectively. Student's t-test showed a significant increase in platelet TBARS levels in cases with neovascularisation as compared to cases without neovascularization (p < 0.05) and healthy controls (p < 0.01). CONCLUSION: The increase in platelet TBARS levels in proliferative Eales' disease is consistent with an emerging view that lipid peroxides may be associated with retinal neovascularisation.

Vasculitis e infección viral / Vasculitis and viral infection

A 17 pacientes con diagnóstico de vasculitis primaria idiopática se les realizó valoración inmunitaria y de anticuerpos antivirales. Se comparó su biometría hemática y sus anticuerpos antivirales con 25 testigos sanos. Todos los pacientes y testigos HIV y HBV resultaron negativos. Se demostró actividad viral por IgM en ocho pacientes: cinco con púrpura vascular, una con enfermedad de Takayazu, una con poliarteritis nodosa y la otra con eritema nodoso. Ningún testigo tuvo IgM positiva. Se detectaron 14 anormalidades en la biometría hemática de los pacientes y en cuatro testigos. Los pacientes exhibieron respuestas de anticuerpos IgG menores que los testigos, alteraciones en subpoblaciones linfocitarias y complejos inmunitarios circulantes. El 47 por ciento de los pacientes tenía infección viral activa, pero la característica predominante fue una respuesta inmunitaria anormal en 82 por ciento

Alteracoes da hemostasia nas vasculites sistemicas / Alterations of hemostasis in systemic vasculitis

Os autores reviram a literatura referente as alteracoes hemostaticas em portadores de vasculites primarias ou naquelas associadas as doencas do colageno. Estes pacientes apresentam uma tendencia trombogenica secundaria a ativacao de plaquetas e a lesao da celula endotelial, muitas vezes associada a alteracoes complexas do sistema de coagulacao e fibrinolise. A ativacao plaquetaria, que se mantem mesmo apos o termino do processo inflamatorio inicial, pode ter um papel central na obstrucao da luz do vaso que se observa na evolucao desses processos.

Immunological status of patients of Eales' disease.

To test the hypothesis that altered immune reactivity to an extraneous agent might lead to primary retinal perivasculitis, a study was undertaken to determine the serum immunoglobulin levels, T lymphocyte subsets, antibody responses to BCG and 'S' antigen, and lymphoproliferative response to mitogens. No difference was observed in these parameters between patients and controls. Both Mantoux positive and negative conditions existed in patients with Eales' disease. Mantoux positive patients showed a higher level of lymphoproliferative response in vitro to PPD than Mantoux positive controls, indicating the presence of two populations among Eales' patients.