RESUMO
OBJECTIVES: We aimed to characterize the clinical and radiological features, and outcomes, of a large cohort of hypertrophic pachymeningitis (HP) patients from a single center. METHODS: We conducted a retrospective study at a tertiary referral center, encompassing patients diagnosed with HP between 2003 and 2022. The diagnosis of HP relied on the identification of thickening of the dura mater via magnetic resonance imaging (MRI) of the brain or spine. RESULTS: We included 74 patients with a mean age of 43.6 ± 14.2 years, of whom 37 (50%) were male. Among them, 32 (43.2%) had an immune-mediated origin, including 21 with granulomatosis with polyangiitis (GPA) (predominantly PR3-ANCA positive), four with systemic lupus erythematosus, three with IgG4-related disease, three with idiopathic HP, and one with rheumatoid arthritis. Non-immune-mediated HP accounted for 45 cases (56.8%). Within this category, 21 (28.4%) were infectious cases, with 14 being Mycobacterium tuberculosis infection (TB-HP), and 21 (28.4%) were malignancy-associated HP. Clinical and MRI characteristics exhibited variations among the four etiological groups. Hypoglycorrhachia was primarily observed in infectious and malignancy-associated HP. Immune-mediated HP was associated with a peripheral pattern of contrast enhancement and the Eiffel-by-night sign. MRI features strongly indicative of TB-HP included leptomeningeal involvement, brain parenchymal lesions, and arterial stroke. MPO-ANCA GPA was associated with a higher prevalence of spinal HP. CONCLUSIONS: Within our cohort, GPA and Mycobacterium tuberculosis emerged as the predominant causes of HP. We identified significant disparities in clinical and radiological features among different etiologies, which could have implications for diagnosis.
RESUMO
The indirect immunofluorescence (IIF) technique for antineutrophil cytoplasmic antibodies (ANCA) detection is subject to substantial differences across laboratories. This study aimed to assess the impact of improvements in the IIF-ANCA technique on the positivity rate of ANCA tests. A cross-sectional study was performed with serum samples from patients with ANCA-associated vasculitis (AAV), autoimmune hepatitis (AIH), and ulcerative colitis (UC). A paired analysis was performed for IIF-ANCA results using the traditional method and a modified protocol after a series of specific adjustments in the technique based on the protocol of IIF-ANCA test performed at a nation-wide private laboratory in Brazil. ANCA specificity was assessed by ELISA for anti-proteinase 3 (PR3) and anti-myeloperoxidase (MPO) antibodies. Sixty-one patients were evaluated. The positivity rate of IIF-ANCA tests at disease presentation performed at the University reference laboratory was 32.3% in AAV, AIH, and UC patients, whereas the positivity rates of IIF-ANCA and ELISA tests in other laboratories were 75.0 and 72.7%, respectively. After modifications in the IIF-ANCA technique, there was a significant increase in the positivity rate (14.8 vs 34.3%; P=0.0002) and in median titers [1/40 (1/30-1/160) vs 1/80 (1/40-1/80); P=0.0003] in AAV, AIH, and UC patients. UC had the highest increment in positive results from 5.3 to 36.8%. There was poor agreement between MPO- or PR3-ANCA and both IIF-ANCA techniques. In conclusion, modifications in the IIF-ANCA protocol led to a significant improvement in its positivity rate and titers.
RESUMO
Abstract Antineutrophil cytoplasmic antibodies (ANCAs) are associated with small vessel vasculitis but their prevalence is not rare in other immune diseases. In lupus nephritis (LN), their pathological role and clinical relevance have been the target of controversial views. We present a case of acute kidney injury and nephrotic syndrome in a young woman with diffuse global proliferative and membranous nephritis on her kidney biopsy, showing a full-house immunofluorescence pattern, very allusive of class IV + V LN, but lacking associated clinical criteria and laboratory findings to support the diagnosis of systemic lupus erythematosus (SLE). Furthermore, the patient presented with high titers of ANCA, steadily decreasing alongside the renal function and proteinuria improvements, with mycophenolate mofetil (MMF) and steroid treatment. The authors believe this is a case of lupus-like nephritis, in which ANCAs are immunological markers, although they are not directly involved in the pathogenesis.
Resumo Os anticorpos anticitoplasma de neutrófilos (ANCAs) estão associados à vasculite de pequenos vasos, no entanto, a sua prevalência não é rara em outras doenças imunológicas. Na nefrite lúpica (LN), o seu papel patológico e relevância clínica têm sido alvo de pontos de vista controversos. Apresentamos um caso de lesão renal aguda e síndrome nefrótica em uma jovem com nefrite proliferativa difusa e membranosa em sua biópsia renal, muito alusivo a NL classe IV + V, com um padrão full house na imunofluorescência, mas sem critérios clínicos e achados laboratoriais para corroborar o diagnóstico de lúpus eritematoso sistêmico (LES). Não obstante, a paciente apresentou títulos elevados de ANCA, que diminuiram progressivamente com a melhoria da função renal e da proteinúria, após tratamento com micofenolato de mofetil (MMF) e esteróide. Os autores acreditam que se trata de um caso de nefrite semelhante à nefrite lúpica, em que os ANCAs são marcadores imunológicos, embora não estejam diretamente envolvidos na patogênese.
RESUMO
We report clinical, serologic, and immunogenetic studies of a set of monozygotic male twin patients who develop autoimmune thyroiditis and vitiligo associated with the HLA-DRB1*04-DQB1*03:02 and HLA-DRB1*03-DQB1*0201 haplotypes. The patients had detectable anti-thyroid and anti-melanocyte autoantibodies. A critical review is presented regarding the role of MHC II molecules linked to clinical manifestations of various autoimmune diseases displayed in a single patient, as is the case in the twin patients reported here.
RESUMO
La vasculitis asociada a propiltiouracilo (PTU) y ANCA es una enfermedad rara con una incidencia de 0,47-0,71 casos por cada 10 000 pacientes. Afecta principalmente a los vasos de menor calibre. Piel, riñones y pulmones son los más afectados. El diagnóstico requiere alta sospecha clínica y se apoya en estudios histopatológicos de las lesiones en piel y serología positiva para ANCA. La suspensión del medicamento suele bastar como manejo de la enfermedad. Mujer de 45 años de edad, con historia de consumo de PTU durante 2 años, desde su diagnóstico de hipertiroidismo; presentando placas purpúricas dolorosas en ambos miembros inferiores.
SUMMARY ANCA vasculitis associated to propylthiouracil (PTU) is a rare condition with an incidence of 0.47-0.71 cases per 100,000 patients. It affects mainly minor caliber blood vessels. The skin, kidneys and lungs are the organs most affected. To make the diagnosis it is necessary to have a high index of suspicion and rely on histopathologic findings of skin lesions plus a positive ANCA test. Stopping the inducing drug is usually necessary to manage this condition. We report the case of a 45-year-old woman who consumed PTU for two years for the treatment of hyperthyroidism presenting with painful purpuric plaques in the lower limbs.
RESUMO
OBJECTIVES: To evaluate the prevalence and meaning of antineutrophil cytoplasmic antibodies (ANCA) positivity in a cohort of IgG4-related disease (IgG4-RD). METHODS: We identified patients with ANCA determination from a retrospective cohort of 69 patients with IgG4-RD. ANCA were measured by indirect immunofluorescence microscopy (IIF) and/or proteinase 3 (PR3)-ANCA and MPO-ANCA by ELISA. IIF patterns were classified as perinuclear (P-ANCA), cytoplasmic (C-ANCA) and atypical (X-ANCA). We compared the ANCA-positive vs the ANCA-negative IgG4-RD group. RESULTS: Out of 69 patients, 31 IgG4-RD patients had an ANCA determination. Four patients with concomitant systemic autoimmune diseases were excluded. We found positive ANCA by IIF in 14 (56%) of 25 patients tested. The most common IIF pattern was C-ANCA in eight (57.1%), followed by dual C-ANCA/X-ANCA in four (28.6%) and P-ANCA and dual C-ANCA/P-ANCA in one each (7.1%). Of the 20 patients with ANCA determination by both IIF and ELISA, four have positive ANCA by ELISA (three for MPO-ANCA and one for PR3-ANCA). Of the two patients with only ELISA determination, one was positive for MPO-ANCA. The prevalence of ANCA positivity by ELISA was 22.7% (5 out of 22 patients). ANCA was more frequent in the Mikulizc/systemic phenotype (42.9%) compared with other phenotypes (P = 0.04). ANCA-positive IgG4-RD patients had more frequently lymph node and kidney involvement, high IgG1 levels and erythrocyte sedimentation rate, and positive antinuclear antibodies. CONCLUSION: ANCA are found in a significant number of patients with IgG4-RD and differed from the ANCA-negative group in terms of clinical and serological features.
Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Doença Relacionada a Imunoglobulina G4/imunologia , Nefropatias/imunologia , Linfonodos/imunologia , Mieloblastina/imunologia , Peroxidase/imunologia , Adulto , Idoso , Doenças da Aorta/imunologia , Doenças Biliares/imunologia , Estudos de Casos e Controles , Feminino , Humanos , Doenças do Aparelho Lacrimal/imunologia , Hepatopatias/imunologia , Masculino , Microscopia de Fluorescência , Pessoa de Meia-Idade , Mieloblastina/metabolismo , Pancreatopatias/imunologia , Peroxidase/metabolismo , Espaço Retroperitoneal , Estudos Retrospectivos , Doenças das Glândulas Salivares/imunologiaRESUMO
Abstract Introduction: The side effects of antithyroid drugs are well known. Antineutrophil cytoplasmic antibody-associated vasculitis is a severe adverse reaction. Most studies evaluating antineutrophil cytoplasmic antibodies related to antithyroid drugs have been carried out with patients treated with propylthiouracil, but less information is available for methimazole. Furthermore, most studies that investigated antineutrophil cytoplasmic antibodies related to antithyroid drugs were conducted on Asian populations. Objective: To evaluate the frequency of antineutrophil cytoplasmic antibodies and antineutrophil cytoplasmic antibodies-positive vasculitis in an adult population of Brazilian patients treated with methimazole. Methods: This was a prospective study. We evaluated patients ≥18 years with Graves' disease who have been using methimazole for at least 6 months (Group A, n = 36); with Grave's disease who had been previously treated with methimazole but no longer used this medication for at least 6 months (Group B, n = 33), and with nodular disease who have been using methimazole for at least 6 months (Group C, n = 13). Results: ANCA were detected in 17 patients (20.7%). Four patients (4.9%) had a strong antineutrophil cytoplasmic antibodies-positive test. The frequency of antineutrophil cytoplasmic antibodies was similar in the groups. When Groups A and B were pooled and compared to Group C to evaluate the influence of Grave's disease, and when Groups A and C were pooled and compared to Group B to evaluate the influence of methimazole discontinuation, no difference was found in the frequency of antineutrophil cytoplasmic antibodies. No difference was observed in sex, age, etiology of hyperthyroidism, anti-TSH receptor antibodies, dose or time of methimazole use between patients with versus without antineutrophil cytoplasmic antibodies. The titers of these antibodies were not correlated with the dose or time of methimazole use. None of the antineutrophil cytoplasmic antibodies-positive patient had clinical event that could potentially result from vasculitis. Conclusion: This clinical study of a Brazilian population shows a considerable frequency of antineutrophil cytoplasmic antibodies in patients treated with methimazole but the clinical repercussion of these findings remains undefined.
Resumo Introdução: Os efeitos adversos de drogas antitireoidianas são conhecidos. Vasculite associada a anticorpos anticitoplasma de neutrófilos é uma reação adversa grave. A maioria dos estudos que avaliam anticorpos anticitoplasma de neutrófilos relacionado a drogas antitireoidianas envolveu pacientes tratados com propiltiouracil, entretanto menos informação se encontra disponível para o metimazol. Além disso, a maioria dos estudos que investigaram anticorpos anticitoplasma de neutrófilos relacionado a drogas antitireoidianas foi conduzida em populações asiáticas. Objetivo: Avaliar a frequência de anticorpos anticitoplasma de neutrófilos e vasculite anticorpos anticitoplasma de neutrófilos-positivo em uma população adulta de pacientes brasileiros tratados com metimazol. Método: Este foi um estudo prospectivo. Avaliamos pacientes ≥ 18 anos com doença de Graves com o uso de metimazol há pelo menos seis meses (Grupo A, n = 36); com doença de Graves previamente tratados com metimazol, mas que não usaram esse medicamento por pelo menos seis meses (Grupo B, n = 33) e com doença nodular em uso de metimazol há pelo menos seis meses (Grupo C, n = 13). Resultado: Anticorpos anticitoplasma de neutrófilos foram detectados em 17 pacientes (20,7%). Quatro pacientes (4,9%) tinham anticorpos anticitoplasma de neutrófilos fortemente positivos. A frequência de anticorpos anticitoplasma de neutrófilos foi semelhante nos grupos. Quando os Grupos A e B foram somados e comparados ao Grupo C para avaliar a influência da doença de Graves, e quando os Grupos A e C foram somados e comparados ao Grupo B para avaliar a influência da interrupção do metimazol, não foi encontrada diferença na frequência de anticorpos anticitoplasma de neutrófilos. Não houve diferença em relação a sexo, idade, etiologia do hipertireoidismo, anticorpos antirreceptor de TSH, dose ou tempo de uso de metimazol entre pacientes com e sem anticorpos anticitoplasma de neutrófilos. Os títulos desses anticorpos não se correlacionaram com dose ou tempo de uso de metimazol. Nenhum paciente anticorpos anticitoplasma de neutrófilos-positivo apresentou evento clínico resultante de vasculite. Conclusão: Este estudo clínico de uma população brasileira apresenta frequência considerável de anticorpos anticitoplasma de neutrófilos em pacientes tratados com metimazol, mas a repercussão clínica desse achado permanece indefinida.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Antitireóideos/efeitos adversos , Doença de Graves/tratamento farmacológico , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Brasil , Doença de Graves/imunologia , Estudos Prospectivos , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/induzido quimicamente , Metimazol/efeitos adversosRESUMO
Resumen La hemorragia alveolar difusa (HAD) masiva es una complicación inusual de los pacientes con vasculitis ANCA, frecuentemente amenaza la vida y está asociada con una mortalidad de hasta el 100%. La información en la literatura acerca del tratamiento en casos refractarios y cuando el paciente se encuentra en diálisis es escasa. Se presenta el caso de un paciente con vasculitis p-ANCA con compromiso renal y pulmonar en el escenario de síndrome pulmón-riñón, con múltiples recaídas de hemorragia alveolar a pesar de tratamiento con corticoide, azatioprina, ciclofosfamida y terapia de recambio plasmático. Se instauró manejo con anticuerpo monoclonal anti CD20 e inmunoglobulina, logrando resolución del episodio de hemorragia alveolar y permaneciendo sin actividad. Se resalta la utilidad del rituximab como estrategia terapéutica en casos refractarios. (Acta Med Colomb 2019; 44: 111-114).
Abstract Massive diffuse alveolar hemorrhage (DAH) is an unusual complication of patients with ANCA vasculitis that frequently threatens life and is associated with mortality up to 100%. In formation in the literature about treatment in refractory cases and when the patient is on dialysis is scarce. The case of a patient with p-ANCA vasculitis with renal and pulmonary involvement in the lung-kidney syndrome scenario, with multiple relapses of alveolar hemorrhage despite treatment with corticosteroid, azathioprine, cyclophosphamide and plasma exchange therapy is presented. Management with anti-CD20 monoclonal antibody and immunoglobulin was estab lished, achieving resolution of the episode of alveolar hemorrhage and remaining without activity. The usefulness of rituximab as a therapeutic strategy in refractory cases is highlighted. (Acta Med Colomb 2019; 44: 111-114).
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Hemorragia , Troca Plasmática , Diálise Renal , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , RituximabRESUMO
INTRODUCTION: The side effects of antithyroid drugs are well known. Antineutrophil cytoplasmic antibody-associated vasculitis is a severe adverse reaction. Most studies evaluating antineutrophil cytoplasmic antibodies related to antithyroid drugs have been carried out with patients treated with propylthiouracil, but less information is available for methimazole. Furthermore, most studies that investigated antineutrophil cytoplasmic antibodies related to antithyroid drugs were conducted on Asian populations. OBJECTIVE: To evaluate the frequency of antineutrophil cytoplasmic antibodies and antineutrophil cytoplasmic antibodies-positive vasculitis in an adult population of Brazilian patients treated with methimazole. METHODS: This was a prospective study. We evaluated patients ≥18 years with Graves' disease who have been using methimazole for at least 6 months (Group A, n=36); with Grave's disease who had been previously treated with methimazole but no longer used this medication for at least 6 months (Group B, n=33), and with nodular disease who have been using methimazole for at least 6 months (Group C, n=13). RESULTS: ANCA were detected in 17 patients (20.7%). Four patients (4.9%) had a strong antineutrophil cytoplasmic antibodies-positive test. The frequency of antineutrophil cytoplasmic antibodies was similar in the groups. When Groups A and B were pooled and compared to Group C to evaluate the influence of Grave's disease, and when Groups A and C were pooled and compared to Group B to evaluate the influence of methimazole discontinuation, no difference was found in the frequency of antineutrophil cytoplasmic antibodies. No difference was observed in sex, age, etiology of hyperthyroidism, anti-TSH receptor antibodies, dose or time of methimazole use between patients with versus without antineutrophil cytoplasmic antibodies. The titers of these antibodies were not correlated with the dose or time of methimazole use. None of the antineutrophil cytoplasmic antibodies-positive patient had clinical event that could potentially result from vasculitis. CONCLUSION: This clinical study of a Brazilian population shows a considerable frequency of antineutrophil cytoplasmic antibodies in patients treated with methimazole but the clinical repercussion of these findings remains undefined.
Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/induzido quimicamente , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Antitireóideos/efeitos adversos , Doença de Graves/tratamento farmacológico , Metimazol/efeitos adversos , Adulto , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Brasil , Feminino , Doença de Graves/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos ProspectivosRESUMO
Abstract INTRODUCTION: Based on reports, infection with Mycobacterium tuberculosis is believed to induce the development of antibodies that are considered to be biological indicators for the diagnosis of some other diseases. However, conflicting results have been published regarding the presence of antineutrophil cytoplasmic antibodies (ANCAs) in patients with tuberculosis. We aim to study the seroprevalence of ANCA in a population of Chinese patients with tuberculosis, which may lead to the misdiagnosis of vasculitic disorders. METHODS: The study was conducted from January 2016 to May 2017 to evaluate the presence of ANCA in 103 Chinese patients using indirect immunofluorescent assay. An enzyme-linked immunosorbent assay was performed for anti-myeloperoxidase (MPO) and anti-proteinase 3 (PR3) detection. RESULTS: Perinuclear ANCA (p-ANCA) was detected in 4.8% (5/103) of patients, whereas cytoplasmic ANCA (c-ANCA) was not detected; 1.9% (2/103) of patients with tuberculosis was positive for anti-MPO antibodies, and none had anti-PR3 antibodies. Both anti-MPO-positive patients were diagnosed with ANCA-associated vasculitides. CONCLUSIONS: ANCA positivity may be more related to vasculitis and immunological disorders than to a M. tuberculosis infection. Therefore, to improve diagnostic accuracy, patients with M. tuberculosis who are ANCA positive should be investigated for concurrent diseases, including the effects of drugs. Therefore, even in tuberculosis epidemic area, ANCA seropositivity, detected by ELISA, is still more suggestive of ANCA-associated vasculitides.
Assuntos
Humanos , Masculino , Feminino , Adulto , Idoso , Tuberculose/sangue , Tuberculose/imunologia , Tuberculose/epidemiologia , Ensaio de Imunoadsorção Enzimática , Estudos Soroepidemiológicos , China/epidemiologia , Estudos Retrospectivos , Técnica Indireta de Fluorescência para Anticorpo , Anticorpos Anticitoplasma de Neutrófilos/sangue , Pessoa de Meia-IdadeRESUMO
Resumen Las vasculitis asociadas a anticuerpos contra el citoplasma del neutrófilo configuran patologías multisistémicas caracterizadas por afectar pequeños vasos. Se ha descrito compromiso de sistema nervioso central en 2-8% de los casos, rara vez al inicio del cuadro clínico. Este corresponde al caso de un hombre de 67 años de edad con vasculitis p-ANCA, quien debutó con proptosis, paquimeningitis y disfagia severa. Posteriormente deterioro renal, necesidad de hemodiálisis, ciclofosfamida y recambio plasmático terapéutico con lo cual alcanza criterios de remisión parcial y egresa de diálisis. Las vasculitis ANCA se relacionan con una amplia gama de signos y síntomas inespecíficos. La edad de aparición, creatinina inicial y requerimiento de diálisis, son factores pronósticos. El inicio oportuno del tratamiento es fundamental, requiriendo por tanto un alto índice de sospecha. (Acta Med Colomb 2018; 43: 107-110).
Abstract Vasculitis associated with antibodies against the neutrophil cytoplasm they form multisystemic pathologies characterized by affecting small vessels. Central nervous system involvement has been described in 2 - 8% of cases, rarely at the beginning of the clinical picture. This corresponds to the case of a 67-year-old man with p-ANCA vasculitis, who debuted with proptosis, pachymeningitis and severe dysphagia. Subsequently, renal deterioration, the need for hemodialysis, cyclophosphamide and therapeutic plasma exchange, with which it reaches criteria of partial remission and dialysis discharge. ANCA vasculitis is associated with a wide range of nonspecific signs and symptoms. The age of onset, initial creatinine and dialysis requirement are prognostic factors. The timely initiation of treatment is fundamental, requiring therefore a high index of suspicion. (Acta Med Colomb 2018; 43: 107-110).
Assuntos
Humanos , Masculino , Idoso , Glomerulonefrite , Meningite , Transtornos de Deglutição , Sistema Nervoso Central , Exoftalmia , Vasculite Associada a Anticorpo Anticitoplasma de NeutrófilosRESUMO
CASE DESCRIPTION: A 16 year-old female who presented with initial ear, nose and throat manifestations who later progressed to severe renal disease, requiring hemodialysis after 11 months of unique laryngeal involvement. CLINICAL FINDINGS: Unilateral vocal cord paralysis without other symptoms or signs, but with positive perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase autoantibodies, followed an unfavorable course months later with rapidly progressive glomerulonephritis. Renal biopsy confirmed an ANCA-associated vasculitis. She was diagnosed with microscopic polyangiitis. TREATMENT AND OUTCOME: High-dose glucocorticoids, intravenous cyclophosphamide, plasma exchange and finally, hemodialysis and renal transplantation. CLINICAL RELEVANCE: In contrast to granulomatosis with polyangiitis (Wegener), ear, nose and throat manifestations in microscopic polyangiitis are uncommon, while involvement of the lungs and kidneys are usual. We present a case with an isolated rare involvement, which progressed to severe disease. This atypical case warns about laryngeal symptoms as initial manifestation of an anti-myeloperoxidase positive systemic vasculitides, and emphasizes the relevance of close observation when unexplained isolated conditions with accompanying evidence of autoimmunity, in this case high levels of specific autoantibodies, are present.
DESCRIPCIÓN DEL CASO: Una mujer de 16 años se presentó inicialmente con manifestaciones otorrinolaringológicas y posteriormente progresó hacia enfermedad renal grave, requiriendo hemodiálisis después de 11 meses de tener exclusivamente afección laríngea. HALLAZGOS CLÍNICOS: parálisis de cuerda vocal unilateral sin otros síntomas ni signos, pero con autoanticuerpos anticitoplasma de neutrófilo (ANCA) con patrón perinuclear y especificidad contra mieloperoxidasa, siguiendo un curso desfavorable meses después con desarrollo de glomerulonefritis rápidamente progresiva. La biopsia renal confirmó una vasculitis asociada con ANCA (VAA). Se diagnosticó entonces como poliangitis microscópica. TRATAMIENTO Y DESENLACE: Glucocorticoides a dosis altas, ciclofosfamida endovenosa, recambio plasmático y finalmente, hemodiálisis y transplante renal. RELEVANCIA CLÍNICA: en contraste con la granulomatosis con poliangitis (Wegener), las manifestaciones otorrinolaringológicas en poliangitis microscópica son poco comunes, mientras que la afección pulmonar y renal es común. Presentamos un caso con afección inusual aislaea, que progresó a enfermedad grave. Este caso atípico enfatiza sobre los síntomas laríngeos como manifestación inicial de una vasculitis antimieloperoxidasa positiva, y subraya la relevancia de una estrecha observación cuando condiciones aisladas inexplicables, que como en este caso se acompañan de evidencia de autoinmunidad manifestado por presencia de niveles altos de autoanticuerpos, se presentan para su atención.
Assuntos
Nefropatias/etiologia , Poliangiite Microscópica/diagnóstico , Paralisia das Pregas Vocais/etiologia , Adolescente , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Autoanticorpos/imunologia , Ciclofosfamida/uso terapêutico , Progressão da Doença , Feminino , Glucocorticoides/uso terapêutico , Humanos , Nefropatias/terapia , Transplante de Rim/métodos , Poliangiite Microscópica/complicações , Poliangiite Microscópica/terapia , Troca Plasmática/métodos , Diálise Renal , Índice de Gravidade de DoençaRESUMO
Abstract Case Description: A 16 year-old female who presented with initial ear, nose and throat manifestations who later progressed to severe renal disease, requiring hemodialysis after 11 months of unique laryngeal involvement. Clinical Findings: Unilateral vocal cord paralysis without other symptoms or signs, but with positive perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase autoantibodies, followed an unfavorable course months later with rapidly progressive glomerulonephritis. Renal biopsy confirmed an ANCA-associated vasculitis. She was diagnosed with microscopic polyangiitis. Treatment and Outcome: High-dose glucocorticoids, intravenous cyclophosphamide, plasma exchange and finally, hemodialysis and renal transplantation. Clinical Relevance: In contrast to granulomatosis with polyangiitis (Wegener), ear, nose and throat manifestations in microscopic polyangiitis are uncommon, while involvement of the lungs and kidneys are usual. We present a case with an isolated rare involvement, which progressed to severe disease. This atypical case warns about laryngeal symptoms as initial manifestation of an anti-myeloperoxidase positive systemic vasculitides, and emphasizes the relevance of close observation when unexplained isolated conditions with accompanying evidence of autoimmunity, in this case high levels of specific autoantibodies, are present.
Resumen Descripción del caso: Una mujer de 16 años se presentó inicialmente con manifestaciones otorrinolaringológicas y posteriormente progresó hacia enfermedad renal grave, requiriendo hemodiálisis después de 11 meses de tener exclusivamente afección laríngea. Hallazgos clínicos: parálisis de cuerda vocal unilateral sin otros síntomas ni signos, pero con autoanticuerpos anticitoplasma de neutrófilo (ANCA) con patrón perinuclear y especificidad contra mieloperoxidasa, siguiendo un curso desfavorable meses después con desarrollo de glomerulonefritis rápidamente progresiva. La biopsia renal confirmó una vasculitis asociada con ANCA (VAA). Se diagnosticó entonces como poliangitis microscópica. Tratamiento y desenlace: Glucocorticoides a dosis altas, ciclofosfamida endovenosa, recambio plasmático y finalmente, hemodiálisis y transplante renal. Relevancia clínica: en contraste con la granulomatosis con poliangitis (Wegener), las manifestaciones otorrinolaringológicas en poliangitis microscópica son poco comunes, mientras que la afección pulmonar y renal es común. Presentamos un caso con afección inusual aislaea, que progresó a enfermedad grave. Este caso atípico enfatiza sobre los síntomas laríngeos como manifestación inicial de una vasculitis antimieloperoxidasa positiva, y subraya la relevancia de una estrecha observación cuando condiciones aisladas inexplicables, que como en este caso se acompañan de evidencia de autoinmunidad manifestado por presencia de niveles altos de autoanticuerpos, se presentan para su atención.
Assuntos
Adolescente , Feminino , Humanos , Paralisia das Pregas Vocais/etiologia , Poliangiite Microscópica/diagnóstico , Nefropatias/etiologia , Troca Plasmática/métodos , Autoanticorpos/imunologia , Índice de Gravidade de Doença , Diálise Renal , Transplante de Rim/métodos , Progressão da Doença , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Ciclofosfamida/uso terapêutico , Poliangiite Microscópica/complicações , Poliangiite Microscópica/terapia , Glucocorticoides/uso terapêutico , Nefropatias/terapiaRESUMO
El levamisol es un antihelmíntico de uso veterinario que ha sido utilizado como aditivo a la cocaína con el objetivo de aumentar los efectos psicotrópicos de dicha sustancia. Esta mezcla produce reacciones como agranulocitosis, vasculitis cutánea y modulación del sistema inmune. Solamente el reconocimiento, tratamiento adecuado y la suspensión del consumo de esta sustancia lleva a la desaparición de las lesiones y sugiere fuertemente el diagnóstico. Presentamos tres casos de síndrome cocaína/levamisol en Medellín y una revisión de la literatura con algunos conceptos de esta enfermedad.
Levamisole, an anthelmintic veterinary product is known to be used as an additive to cocaine that enhances the psychotropic effects of this substance. This mixture leads to reactions such as agranulocytosis, skin vasculitis and modulation of the immune system. Only the proper recognition, treatment of this entity and discontinuation of this substance consumption, leads to the disappearance of lesions; strongly suggesting the diagnosis. We present three cases of cocaine/levamisole syndrome in the city of Medellin and a review of the literature with some concepts related to this pathology.
RESUMO
Granulomatosis with polyangiitis (GPA) is associated with a broad range of clinical manifestations including renal disease. It is a systemic vasculitis that is rarely encountered in children. We present a 14-year-old girl who suffered from pharyngitis 1 week before admittance to hospital. She was admitted for macroscopic hematuria and oliguria, under the possibility of nephritic syndrome. Renal failure with rapidly progressive glomerulonephritis occurred within 24 hours. Immunologic tests showed the presence of type-C anti-neutrophil cytoplasmic antibodies (c-ANCA with antiproteinase 3 specificity) and renal biopsy revealed pauci-immune crescentic focal necrotizing glomerulonephritis. Treatment including methylprednisolone and cyclophosphamide intravenous pulses allowed renal recovery after 3 weeks. The clinical, hematological, and biochemical parameters improved substantially, achieving remission. Granulomatosis with polyangiitis, although rare in children, should be considered in the above clinical scenario. This case underlines that knowledge of renal histology diagnosis and early aggressive immunosuppressive therapy are essential for the management of these patients.
RESUMO
Muitas vasculites sistêmicas acometem o trato respiratório. O acometimento mais frequente é o do parênquima pulmonar pelas vasculites de pequenos vasos ANCA associadas (antineutrophil cytoplasmic antibodies), que incluem a granulomatose com poliangite (GPA, antiga granulomatose de Wegener), a poliangite microscópica (MPA), granulomatose eosinofílica com poliangite (EGPA, antiga doença de Churg-Strauss). Este artigo propõe-se a revisar o tratamento dessas doenças de acordo com recomendações da liga europeia de combate ao reumatismo (EULAR), com base no estádio e na atividade de doença, incluindo as terapia usadas na indução e na manutenção de remissão, assim como nos casos refratários ao tratamento convencional
There are many vasculitis that affect the respiratory tract. The pulmonary parenchyma is the most frequently involved and occurs mainly in ANCA associated small vessel vasculitis (antineutrophil cytoplasmic antibodies), which include granulomatosis with polyangitis (GPA, formely Wegener's granulomatosis), microscopic polyangitis (MPA), eosinophilic granulomatosis with polyangitis (EGPA formely Churg-Strauss Syndrome). This article reviews the treatment of them, according to EULAR (European League Against Rheumatism) recommendations, which are based on stage and severity of disease, including induction and maintance therapy, and therapy of refractory disease
Assuntos
Humanos , Masculino , Feminino , Anticorpos Anticitoplasma de Neutrófilos/uso terapêutico , Vasculite/terapia , PneumopatiasRESUMO
O uso do propiltiouracil (PTU) está associado ao desenvolvimento de diferentes auto-anticorpos, entre eles anticorpos anticitoplasma de neutrófilos (ANCA), que estão envolvidos na patogênese das vasculites sistêmicas ANCA-associadas (VSAA). Será relatado o caso de um paciente do sexo feminino, de 46 anos, que apresentou vasculite cutânea durante o uso de PTU como terapêutica para doença de Graves. O ANCA com padrão perinuclear (p-ANCA) foi positivo em títulos maior ou igual 1/320, porém anticorpos antimielo-peroxidase (MPO) não foram detectados. A biópsia de pele revelou uma vasculite leucocitoclástica. Houve melhora clínica em dez dias após a retirada do PTU e optou-se pelo iodo radioativo (I131) para o tratamento do hipertireoidismo. O p-ANCA manteve-se positivo em títulos maior ou igual 1/320 em duas medidas, realizadas oito meses e quatro anos após a suspensão do PTU.
The use of propylthiouracil (PTU) is associated with the development of different auto-antibodies, amongst them are antineutrophil cytoplasmic antibodies (ANCA) that are involved in the pathogenesis of ANCA associated systemic vasculitis. The case of a 46-years old woman who presented cutaneous vasculitis when taking PTU for Graves' disease is reported. Perinuclear ANCA (p-ANCA) was positive with titer > 1/320, but anti-myeloperoxidase antibodies were not detected. Skin biopsy showed leucocytoclastic vasculitis. The patient improved within ten days after withdrawing PTU and the resolution of hyperthyroidism was achieved with radioiodine (131I). The p-ANCA test remained positive > 1/320 eight months and four years after PTU withdrawal.
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Anticorpos Anticitoplasma de Neutrófilos , Doenças do Sistema Endócrino , Hipertireoidismo , Propiltiouracila , Vasculite , Vasculite Leucocitoclástica CutâneaRESUMO
Vasculite é a inflamação da parede dos vasos. Pode variar em gravidade desde doença autolimitada de um único órgão até doença grave com risco de morte por falência de múltiplos órgãos. Existem várias causas, embora só se apresente por poucos padrões histológicos de inflamação vascular. Vasos de qualquer tipo e em qualquer órgão podem ser afetados, resultando em ampla variedade de sinais e sintomas. Diferentes vasculites com apresentações clínicas indistinguíveis têm evolução e tratamento muito diferentes. Essa condição representa desafio para o médico, incluindo classificação, diagnóstico, exames laboratoriais pertinentes, tratamento e seguimento adequado. Neste artigo são revistos a classificação, a etiologia, a patogênese e os critérios diagnósticos das vasculites cutâneas.
Vasculitis is an inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple-organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have very different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. This article reviews the classification, etiology, pathology and diagnostic criteria of cutaneous vasculitis.