Granulomatosis con poliangeítis. A propósito de un caso

La granulomatosis con poliangeítis es una vasculitis sistémica, necrosante y granulomatosa que afecta el tracto respiratorio superior e inferior y los riñones. Se presenta el caso de una paciente blanca, de 59 años de edad, hipertensa y fumadora inveterada, que ingresó por presentar síntomas constitucionales, orinas turbias y hemáticas, así como creatininemia elevada con valor previo normal. Con impresión diagnóstica inicial de infección del tracto urinario se indica antibioticoterapia. Después del tercer día de iniciado el tratamiento, mejoraron los síntomas constitucionales y, de forma progresiva, comenzó con manifestaciones multiorgánicas (renal, respiratoria, cutánea y musculoesquelética) sugestivas de vasculitis sistémica. Como elementos significativos, en exámenes complementarios se detectaron azoados y reactantes de fase aguda aumentados, sedimento urinario activo, imagen nodular en vértice pulmonar derecho y microhemorragias multifocales en ambos campos pulmonares, detectados mediante tomografía computarizada y c-ANCA altamente positivo. Se indicó tratamiento inmunosupresor y citotóxico potente, obteniéndose control inicial de las manifestaciones graves de la enfermedad. Evolutivamente desarrolló complicaciones propias de la entidad y secundarias al tratamiento médico, que la hicieron tributaria de terapia de sustitución renal, falleciendo a los dos años de recibir hemodiálisis iterada. El cuadro clínico, unido a los estudios imagenológicos e inmunológicos (c-ANCA), fueron elementos claves para realizar el diagnóstico.

Granulomatosis with polyangiitis is a systemic, necrotizing, granulomatous vasculitis that affects the upper and lower respiratory tract and kidneys. The case of a 59-year-old white female patient, who is hypertensive and inveterate smoker, is presented; she was admitted for presenting constitutional symptoms, turbid and bloody urine, as well as elevated creatininemia with a normal previous value. With the initial diagnostic impression of urinary tract infection, antibiotic therapy was indicated. After the third day of starting treatment the constitutional symptoms improved, and progressively began with multiorgan manifestations (renal, respiratory, skin and musculoskeletal) suggestive of systemic vasculitis. As significant elements, complementary examinations detected increased nitrogen and acute phase reactants, active urinary sediment, nodular image in the right lung apex and multifocal microhemorrhages in both lung fields through computed tomography, and highly positive c-ANCA. Potent immunosuppressive and cytotoxic treatment was indicated, obtaining initial control of severe manifestations of the disease. Evolutionarily she developed complications specific to the entity and secondary to medical treatment which made her subject to renal replacement therapy, dying two years after receiving iterated hemodialysis. The clinical characteristics, together with the imaging and immunological studies (c-ANCA) were key elements to make the diagnosis.

Wegener’s Granulomatosis: Are We Still Missing It.

We report the case of an 18-year-old female who was mis-diagnosed as a smear-negative pulmonary tuberculosis and advised standard antituberculosis treatment. She later presented with clinio-radiological worsening and thrombosis of superficial veins of the lower extremity. Cytoplasmic anti-neutrophil cytoplasmic antibody and computed tomographyguided lung biopsy confirmed the diagnosis of Wegener’s granulomatosis. The rare association of superficial vein thrombosis with lung manifestation is highlighted here as also the need for a high index of clinical suspicion to avoid a missed or delayed diagnosis.

Granulomatosis con poliangeitis (de Wegener) asociada a dengue / Granulomatosis with polyangiitis (Wegener's) associated with dengue

Introducción: el dengue produce una activación del sistema inmune y en los pacientes conocidos portadores de enfermedades autoinmunes puede ser responsable de la activación de la enfermedad así como en los no conocidos puede revelar la enfermedad, por lo tanto ante un paciente con dengue con una presentación no habitual las enfermedades autoinmunes deben ser descartadas. Objetivo: presentar un paciente con dengue asociado a la activación de una vasculitis sistémica. Caso clínico: varón de 69 años, ingresó con cefalea, fiebre y mialgias en febrero 2012, el dengue fue sospechado y confirmado por serología IgM positiva. Debido a la persistencia de la fiebre, aparición de úlceras bucales, traqueales y nefritis asociada, se sospechó la poliangeitis granulomatosa (de Wegener) con ANCA-C positiva. Recibió tratamiento con pulsos de metilprednisolona y ciclofosfamida con regresión de las lesiones. Un mes después del alta fallece por insuficiencia respiratoria aguda secundaria a una estenosis subglótica secuela de la úlcera traqueal.

Introduction: Dengue produce an activation of the immune system in patients known to be carriers of autoimmune diseases, may be responsible for the activation of the disease as well as in unknown cases can reveal the disease itself, therefore a patient with dengue and unusual presentation, autoimmune disease should be ruled out. Objective: To present a patient with dengue associated with the activation of a systemic vasculitis. Case report: A 69 year old man was admitted with headache, fever and myalgia in February 2012, dengue was suspected and clinically confirmed by positive IgM serology. Due to persistent fever, oral and tracheal ulcers and associated nephritis, granulomatous polyangiitis (Wegener) with positive cANCA was suspected. She was treated with methylprednisolone and cyclophosphamide pulses with regression of lesions. One month after discharge, patient die from acute respiratory failure secondary to subglottic stenosis due to previous traqueal ulcer.

ANCA-Negative Wegener's Granulomatosis with Multiple Lower Cranial Nerve Palsies

Wegener's granulomatosis (WG) is a systemic vasculitis affecting small and medium-sized vessels with granulomatous formation. Though it is known for respiratory tract and kidney involvement, neurologic manifestation has been also reported. Herein we report a patient who suffered pansinusitis with multiple lower cranial nerve palsies but reached remission by immunosuppressant after the diagnosis of WG. A 54-yr-old female visited with headache, hearing difficulty, and progressive bulbar symptoms. She experienced endoscopic sinus surgeries due to refractory sinusitis. Neurologic examination revealed multiple lower cranial nerve palsies. Vasculitic markers showed no abnormality. Nasal biopsy revealed granulomatous inflammation and vasculitis involving small vessels. Given cyclophosphamide and prednisolone, her symptoms were prominently improved. WG should be considered in the patient with multiple cranial nerve palsies, especially those with paranasal sinus disease. Because WG can be lethal if delayed in treatment, prompt immunosuppressant is warranted after the diagnostic tissue biopsy.

Immunofluorescence (IF) Pattern, Autoantigens of Anti-neutrophil Cytoplasmic Antibodies (ANCA) and Their Clinical Associations / Jurnal Sains Kesihatan Malaysia

Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies directed against primary granules of neutrophils and monocytes’ lysosomes. In general, c-ANCA is strongly associated with vasculitic disorders mainly in ANCA-associated systemic vasculitis (AASV). p-ANCA have been identified in several diseases such as primary (AASV) and secondary vasculitis such as collagen vascular diseases, rheumatoid arthritis and inflammatory bowel diseases given the term ‘ANCA-associated disease.’ The objective of this study was to determine the rate of ANCA positivity by indirect immunofluorescent (IF) and enzyme linked immunosorbent assay (ELISA) and its association with AASV and ANCA associated diseases. Serum from patients with history suspicion of systemic vasculitis were tested for ANCA by IF. Those samples positive for ANCA by IF were further tested for antibodies against myeloperoxidase (MPO) and proteinase 3 (PR3) using the ELISA. Clinical data from medical records were obtained and analyzed. Of 468 samples, a total of 110 were positive for ANCA by IF. IF results showed a p-ANCA pattern in 96 patients and c-ANCA in 14. Of 110 IF positive ANCA, 45 patients were positive by ELISA. Seventeen were positive for MPO-ANCA, 9 were PR3-ANCA positive and 19 were both MPO and PR3 positive. Only 2 patients were classified AASV ie Wegener granulomatosis and the other with microscopic polyangiitis. The remaining patients (n = 108) may be classified as ANCA associated diseases. Our study showed that p-ANCA (87.3%) was the more common ANCA pattern and 40.9% of IF positive samples were positive for PR3- and MPOANCA.

Anticorpos contra o citoplasma de neutrófilos / Antineutrophil cytoplasmic antibodies

Os auto-anticorpos antiantígenos citoplasmáticos de neutrófilos (ANCA) têm importante associação ao diagnóstico e possível monitoramento de uma significante parcela de doenças auto-imunes. A Proteinase 3 é o principal antígeno presente no tipo demarcação citoplasmática granular difusa (c-ANCA), e a Mieloperoxidase na marcação perinuclear (p-ANCA) em ensaios de imunofluorescência indireta (IFI) em neutrófilos fixados em etanol. A vasculite sistêmica compreende uma série de síndromes caracterizadas por dividir uma base histopatológica comum: inflamação nos vasos sangüíneos resultando em obstrução vascular com subseqüente isquemia e enfartamento tissular. A vasculite constitui um grupo heterogêneo de doenças que possuem como característica comum à inflamação destrutiva da parede de vasos sangüíneos. O papel potencializador de ANCA sobre as lesões é descrito em estágios iniciais de vasculite sistêmica pelo fato deste promover o recrutamento e adesões entre neutrófilos e células endoteliais.O presente trabalho teve como objetivo enfocar o papel do c-ANCA e p-ANCA no diagnóstico laboratorial de Vasculite Sistêmica e o levantamento de exames efetuados no período de 16/04/04 a 20/04/06 para estes mesmos marcadores no laboratório de ImunologiaClínica do Hospital Universitário Polydoro Ernani de São Thiago. Neste levantamento pudemos constatar uma reduzida parcela de resultados positivos para c-ANCA (15%) e p-ANCA (39%), fato este devido à larga gama de doenças com sintomatologias semelhantes às doenças relacionadas à ANCA, possuindo, porém ANCA negativo.A avaliação clínica é importante no manejo de doenças auto-imunes, mas o laboratório representa papel decisivo na avaliação dessas doenças. Testes laboratoriais auxiliam ao estabelecer o diagnóstico, na monitorização do curso da doença, na predição de sua evolução,na decisão acerca da terapêutica, na avaliação da resposta à terapia e também para o estudo da etiologia ou patogênese das doenças auto-imunes

Wegener's Granulomatosis Involving Nasal Cavity / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Wegener's granulomatosis is a chronic multisystem vasculitis. The otolaryngologist has an important role in the early diagnosis of Wegener's granulomatosis because of the high incidence of nasal involvement. In this study, we analyze seven cases of Wegener's granulomatosis involving nasal cavity and describe clinical features, laboratory findings, treatments and outcomes. MATERIALS AND METHOD: Selected for this study were 7 inpatients with Wegener's granulomatosis treated at the department of otolaryngology of Korea University Medical Center from January, 1985 and December, 2002. Their clinicopathologic records were reviewed retrospectively. RESULTS: The nasal manifestations included nasal crusting, nasal obstruction, serous and bloody nasal discharge, septal perforation and saddle nose. All patients showed positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) and an elevated erythrocyte sedimentation rate (ESR). Wegener's granulomatosis was confirmed by the results of nasal mucosal biopsy performed in all patients. CONCLUSION: If multiple and large biopsy sites were selected properly, the diagnostic efficacy of nasal biopsy in Wegener's granulomatosis may increase and it may help early diagnosis since nasal mucosa is the most commonly affected site in Wegener's granulomatosis and nasal cavity is an easily accessible biopsy site.

Pulmonary Cavitary Lesions Associated with High Fever / 결핵

No abstract available.