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1.
Medicine (Baltimore) ; 98(51): e18139, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31860960

RESUMO

RATIONALE: Rituximab is recommended to induce remission of severe granulomatosis with polyangiitis (GPA). Plasma exchange (PE) may be considered in the setting of rapidly progressive glomerulonephritis (RPGN) with a serum creatinine increase of more than 5.6 mg/dl or diffuse alveolar hemorrhage (DAH). However, there are no sufficient studies on combination therapy with rituximab and PE in GPA. PATIENT CONCERNS: A 23-year-old woman was admitted with fever, abdominal pain, and diarrhea on suspicion of infectious colitis. Colonoscopy showed hemorrhagic colitis and antibiotic treatment was ineffective. Physical examination revealed episcleritis and skin lesions similar to Janeway lesions or Osler nodes on her palms and soles. Transesophageal echocardiogram (TEE) revealed mitral valve vegetation mimicking infective endocarditis. However, no pathogen was grown in the blood culture. Ten days after admission, blood-tinged sputum and respiratory distress developed. Imaging studies of lung, bronchoscopy, and bronchoalveolar lavage indicated DAH. Moreover, serum creatinine levels rapidly increased from 0.8 mg/dl to 6.1 mg/dl with proteinuria. DIAGNOSIS: The patient was diagnosed with GPA and non-infectious endocarditis, DAH, and RPGN, based on a biopsy which revealed pauci-immune crescentic glomerulonephritis with granuloma and leukocytoclastic vasculitis and antineutrophil cytoplasmic antibodies against proteinase 3- positivity. INTERVENTIONS: Initial methylprednisolone pulse therapy (1 g daily for 3 days) proved unsuccessful. After initiating PE, creatinine levels began to slowly decline, but DAH continued to deteriorate. Rituximab combined with PE therapy was considered. We performed PE every 2 to 3 days for 5 total treatments combined with rituximab (375 mg/m, once weekly for 4 weeks). OUTCOMES: After the combination treatment of rituximab and PE, alveolar hemorrhage stopped. Chest X-ray and laboratory data, including serum creatinine and hemoglobin, notably improved. Mitral valve vegetation was no longer observed in follow-up TEE. GPA remained stable with low dose prednisolone and immunosuppressants over a follow-up period of 5 years. LESSONS: This case suggests that the use of rituximab and concurrent PE may represent a promising combination for severe and refractory GPA.


Assuntos
Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Troca Plasmática/métodos , Rituximab/uso terapêutico , Anticorpos Monoclonais/uso terapêutico , Biópsia por Agulha , Colite/diagnóstico , Colite/etiologia , Colonoscopia/métodos , Terapia Combinada , Esquema de Medicação , Serviço Hospitalar de Emergência , Feminino , Seguimentos , Granulomatose com Poliangiite/diagnóstico , Humanos , Imuno-Histoquímica , Medição de Risco , Índice de Gravidade de Doença , Fatores de Tempo , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Adulto Jovem
2.
Immunol Med ; 42(2): 94-98, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31469613

RESUMO

Giant cell arteritis (GCA) is a type of large vessel vasculitis, and it involves the aorta, large vessels and terminal branches of the external carotid artery, especially the temporal artery. Temporal artery biopsy is a simple tool for the diagnosis of vasculitis, however, the histopathological findings do not always differentiate between the small-vessel vasculitis and GCA. We report the case of 72-year-old male who initially had a clinical diagnosis of GCA, then in the course of treatment, diagnostic histopathological approach revealed the necrotizing vasculitis with bronchocentric granulomatosis in the inflammatory nodule of the lung. The manifestations of patients with systemic vasculitis represent the disorders of multiple organ systems thus are diverse and may vary through the course of the disease. Presentation of unexpected features such as insufficient response to antibiotics, sinusitis, runny nose, discomfort of frontal region or pachymeningitis which anticipates re-evaluation of systemic vasculitis that may lead us to an appropriate diagnosis and the treatment.


Assuntos
Arterite de Células Gigantes/diagnóstico , Granulomatose com Poliangiite/diagnóstico , Idoso , Biópsia , Diagnóstico Diferencial , Imagem de Difusão por Ressonância Magnética , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/patologia , Arterite de Células Gigantes/terapia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/patologia , Granulomatose com Poliangiite/terapia , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Masculino , Radiografia Torácica , Rinite/etiologia , Sinusite/etiologia , Artérias Temporais/patologia , Tomografia Computadorizada por Raios X
3.
BMJ Case Rep ; 12(7)2019 Jul 08.
Artigo em Inglês | MEDLINE | ID: mdl-31289155

RESUMO

A 22-year-old female patient was admitted to hospital after being referred from the oral medicine clinic where she had been seen for persistent gingivitis and mouth ulcers. She described an insidious history of persistent fevers, dry cough and unexplained weight loss over 4-6 weeks. Imaging showed extensive bilateral pulmonary nodules with mediastinal lymphadenopathy and two lesions in the pancreas. MRI revealed these lesions to be well-defined fluid-filled cysts in the tail of the pancreas, without features of malignancy. Core biopsies taken from her lung nodules demonstrated features of vasculitis with granulomata. This was consistent with her positive immunology for c-antinuclear cytoplasmic antibodies and proteinase-3, which were sent after her fever failed to settle with antibiotic treatment. In keeping with a diagnosis of vasculitis, the patient showed a significant clinical and biochemical response to intravenous methylprednisolone and high-dose daily prednisolone thereafter.


Assuntos
Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Linfadenopatia/patologia , Administração Intravenosa , Anticorpos Anticitoplasma de Neutrófilos/sangue , Diagnóstico Diferencial , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/diagnóstico por imagem , Granulomatose com Poliangiite/metabolismo , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/patologia , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Mieloblastina/sangue , Pâncreas/diagnóstico por imagem , Pâncreas/patologia , Resultado do Tratamento , Adulto Jovem
4.
PLoS One ; 14(7): e0218705, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31291263

RESUMO

AIMS: Cytomegalovirus (CMV) infection under immunosuppression sometimes causes death. This study aimed to elucidate risk factors for CMV infection in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). METHODS: Patients with AAV who underwent remission induction treatment at Okayama University Hospital between 2006 and 2016 were retrospectively analyzed. The primary outcome was the development of CMV infection within 3 months. RESULTS: Of the 111 patients, 13 (11.7%) patients developed CMV infection. Patients with CMV infection were older (p = 0.030) and had a higher body mass index (p = 0.029) in comparison to those without CMV infection. A higher proportion had a severe form (p = 0.001) and granulomatosis with polyangiitis (GPA) (p = 0.001), as well as a higher Birmingham Vasculitis Activity Score (p = 0.018) and C-reactive protein (p = 0.018) levels at baseline. Using logistic regression analysis, severe form and GPA were independent risk factors (odds ratio [OR] = 9.68, 95% confidence interval [CI] = 1.92-60.23, and OR = 7.46, 95% CI = 1.46-47.60, respectively). In addition, patients with CMV infection were more likely than those without infection to be glucocorticoid-related diabetes mellitus (p = 0.025). CONCLUSION: Our study highlights disease severity and subgroups of AAV as risk factors for CMV infection.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Infecções por Citomegalovirus/imunologia , Citomegalovirus/imunologia , Granulomatose com Poliangiite/imunologia , Infecções Oportunistas/imunologia , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Índice de Massa Corporal , Proteína C-Reativa/metabolismo , Citomegalovirus/isolamento & purificação , Citomegalovirus/patogenicidade , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/etiologia , Infecções por Citomegalovirus/virologia , Feminino , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Humanos , Imunossupressores/administração & dosagem , Imunossupressores/efeitos adversos , Japão , Modelos Logísticos , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/efeitos adversos , Pessoa de Meia-Idade , Infecções Oportunistas/diagnóstico , Infecções Oportunistas/etiologia , Infecções Oportunistas/virologia , Indução de Remissão , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença
5.
Yonsei Med J ; 60(8): 705-712, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31347324

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophilia, and eosinophil-rich granuloma of the respiratory tracts. EGPA has three clinical and histological stages. The first is an allergic stage composed of asthma and sinusitis, and the second is an eosinophilic stage characterised by peripheral hypereosinophilia and intra-organ infiltration of eosinophils. The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus.


Assuntos
Grupo com Ancestrais do Continente Asiático , Granulomatose com Poliangiite/patologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite/classificação , Granulomatose com Poliangiite/terapia , Granulomatose com Poliangiite/virologia , Vírus da Hepatite B/fisiologia , Humanos , Prognóstico , República da Coreia
6.
PLoS One ; 14(6): e0218272, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31216309

RESUMO

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), are autoimmune conditions associated with small vessel inflammation. Earlier studies indicate that complement activation via the alternative pathway plays a major role in the pathogenesis. In this study we have investigated if ANCA-activation of neutrophils from AAV patients leads to activation of the alternative complement pathway. C5a-primed neutrophils (PMN) from 10 AAV patients and 10 healthy controls (HC) were stimulated with PMA or IgG purified from PR3-ANCA positive patients (ANCA IgG). The supernatants were analyzed for release of complement proteins and markers of different granules by ELISA, and release of microparticles (MP) by flow cytometry. The ability of the supernatants to activate the alternative complement pathway was determined by incubation with normal serum and C3bBbP and C5a were measured by ELISA. MP were analyzed by flow cytometry and removed by centrifugation. The supernatants from the AAV patients' neutrophils produced significantly more C3bBbP compared with HCs (p = 0.0001). C3bBbP levels correlated with the number of MP. After removal of MP from the supernatants, alternative pathway activation was significantly lower. This study shows that primed and ANCA-stimulated neutrophils from AAV patients have a greater ability to activate the alternative complement pathway compared to primed neutrophils from healthy controls. This finding emphasizes the role of complement in the pathogenesis of AAV - underlining the therapeutic potential of C5a and other complement blockade.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Inflamação/imunologia , Neutrófilos/imunologia , Idoso , Animais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/sangue , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Ativação do Complemento/efeitos dos fármacos , Ativação do Complemento/imunologia , Complemento C5a/imunologia , Ensaio de Imunoadsorção Enzimática , Feminino , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/imunologia , Granulomatose com Poliangiite/patologia , Humanos , Imunoglobulina G/administração & dosagem , Imunoglobulina G/imunologia , Inflamação/sangue , Inflamação/patologia , Masculino , Poliangiite Microscópica/sangue , Poliangiite Microscópica/imunologia , Poliangiite Microscópica/patologia , Neutrófilos/patologia
7.
Rheumatol Int ; 39(7): 1269-1277, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31073659

RESUMO

To present a case of prostate involvement (PI) in granulomatosis polyangiitis (GPA) and analyse related published reports. We employed the following keywords for retrieving reports indexed by MEDLINE/PubMed and/or Scopus: "granulomatosis with polyangiitis", "Wegener granulomatosis" and "prostate involvement". Additional searches were performed through Google Scholar and HINARI. All cases that fulfilled the American College of Rheumatology criteria for GPA, standards of Chapel Hill Consensus Conference, and did not match with exclusion criteria were analysed and summarised. A 35-year-old man presented with complaints of stuffy nose, difficulty breathing through the nose, swelling and pain in the left half of the nose, low-grade fever, and discomfort. The nasal mucosal biopsy did not reveal any specific changes. During the inpatient treatment, he developed eye redness, tearing, dysuria, and decreased urinary stream. Prostate-specific antigen (PSA) was elevated (2.81 µg/L; normal values ≤ 1.4 µg/L for males below 40 years). Prostate biopsy findings were consistent with diagnosis of GPA, which was confirmed by detecting elevated anti-PR3 antibodies (4.1 IU; normal values < 1.0 IU). We analysed our case in view of the clinical course of 45 published cases of PI in GPA. PI in GPA is a rare clinical manifestation of the vasculitis. Patients with atypical clinical symptoms of GPA are at risk of delayed diagnosis. The awareness of variable clinical presentations of GPA, particularly specific affection of the prostate gland, is crucial for timely diagnosis.


Assuntos
Granulomatose com Poliangiite/diagnóstico , Próstata/patologia , Adulto , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/patologia , Humanos , Masculino , Antígeno Prostático Específico/sangue
8.
Rheumatol Int ; 39(7): 1279-1284, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31139949

RESUMO

Granulomatosis with polyangiitis (GPA) commonly presents with glomerulonephritis and inflammation of upper and lower respiratory tracts. It can also involve other organs including those of the urinary tract. The involvement of the urethra is very rarely reported. We present a case of GPA in a patient who had recurrent urinary tract infections and an acute bladder outlet obstruction due to a urethral thickening by GPA. In this report, we discuss urethral involvement with GPA. The incidence of such involvement, as with other urinary tract organs, might be underestimated. It can affect both sexes, with male predominance, and can occur at any age. It responds to standard GPA medical treatment but may require surgical intervention. Rheumatologists should be aware of this limited form of GPA as early recognition and treatment can decrease the risk of complications.


Assuntos
Granulomatose com Poliangiite/diagnóstico , Uretra/patologia , Feminino , Granulomatose com Poliangiite/patologia , Humanos , Pessoa de Meia-Idade
9.
BMJ Case Rep ; 12(5)2019 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-31092487

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis characterised by atopic manifestations, inflammation of small-sized to medium-sized arteries and veins, hypereosinophilia and tissue infiltration with eosinophils. Cardiac complications occur most commonly in the absence of antineutrophil cytoplasmic antibodies. Cardiac complications include coronary arteritis (rare), pericarditis, myocarditis, endocardial fibrosis (Loeffler's endocarditis) and intracavitary thrombosis of the left or right ventricle. This is the first reported case of large non-infectious valvular vegetations associated with EGPA.


Assuntos
Valva Aórtica/patologia , Granulomatose com Poliangiite/diagnóstico , Valva Mitral/patologia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Dispneia/etiologia , Ecocardiografia Transesofagiana , Evolução Fatal , Granulomatose com Poliangiite/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgia
10.
BMJ Case Rep ; 12(4)2019 Apr 24.
Artigo em Inglês | MEDLINE | ID: mdl-31023740

RESUMO

Gingival pathology is a daily presentation, however a small number of systemic conditions can manifest similar to a common gingival condition and have fatal results. Dentist referred 56-year-old woman to Oral and Maxillofacial Surgery department with a 2-week medical history of gingival bleeding not responding to local measures. Biopsy showed eosinophilic infiltrate and vasculitis, and blood tests showed positive markers including cytoplasmic antineutrophil cytoplasmatic antibodies. Granulomatosis with polyangiitis is a rare disease affecting the respiratory tract, blood vessels and kidneys. Oral lesions are rarely the primary presenting feature. When left untreated, most cases are fatal within a year of diagnosis. The diagnosis can only be made when certain criteria are found, including granular oral lesions exhibiting an eosinophilic inflammatory infiltrate on biopsy. With 5% of cases showing intraoral lesions as the primary feature, it is essential that dentists have the knowledge of this rare disease to refer and not to treat as a common gingival condition.


Assuntos
Odontólogos/educação , Doenças da Gengiva/diagnóstico , Granulomatose com Poliangiite/patologia , Úlceras Orais/etiologia , Assistência ao Convalescente , Antibacterianos/uso terapêutico , Anticorpos Anticitoplasma de Neutrófilos/metabolismo , Biópsia , Diagnóstico Diferencial , Eosinófilos/patologia , Feminino , Células Gigantes de Corpo Estranho/patologia , Doenças da Gengiva/patologia , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/sangue , Granulomatose com Poliangiite/tratamento farmacológico , Hemorragia/diagnóstico , Hemorragia/etiologia , Humanos , Pessoa de Meia-Idade , Úlceras Orais/diagnóstico , Úlceras Orais/tratamento farmacológico , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Doenças Raras , Resultado do Tratamento , Combinação Trimetoprima e Sulfametoxazol/administração & dosagem , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico
11.
Tidsskr Nor Laegeforen ; 139(7)2019 Apr 09.
Artigo em Norueguês, Inglês | MEDLINE | ID: mdl-30969044

RESUMO

BACKGROUND: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, has a predilection for the upper airways, lungs and kidneys. However, any other organ can be affected. Although cutaneous lesions are common, they have only rarely been reported as a primary manifestation of the disease. CASE PRESENTATION: We present a case of a teenage boy with pyoderma gangrenosum-like ulcerations of the neck and face. Anti-neutrophil cytoplasmic antibody with antigen specificity for proteinase 3 (PR3-ANCA) was detected. In the absence of other symptoms and organ manifestations, the ulcerations were still considered to be pyoderma gangrenosum. The ulcers started to heal during treatment with corticosteroids and infliximab. One month later the patient developed sinusitis, and eventually lost vision in his left eye. The diagnosis was changed to GPA and he started treatment with methylprednisolone, rituximab and cyclophosphamide with good response on vision, sinusitis and ulcerations. INTERPRETATION: Recognition of this rare skin presentation of GPA is essential, to prevent delays in diagnosis and treatment that can lead to organ damage.


Assuntos
Face/patologia , Granulomatose com Poliangiite , Pescoço/patologia , Úlcera Cutânea/etiologia , Adolescente , Fármacos Dermatológicos/administração & dosagem , Fármacos Dermatológicos/uso terapêutico , Diagnóstico Diferencial , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/patologia , Humanos , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/uso terapêutico , Infliximab/administração & dosagem , Infliximab/uso terapêutico , Masculino , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Pioderma Gangrenoso/diagnóstico , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Úlcera Cutânea/tratamento farmacológico , Úlcera Cutânea/patologia
12.
BMJ Case Rep ; 12(3)2019 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-30872335

RESUMO

Granulomatosis with polyangiitis (GPA) was diagnosed in a patient with a 16-month history of IgG4-related lung disease that spontaneously became asymptomatic. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA) was positive at the time of diagnosis of IgG4-related disease (IgG4-RD), but there was no vasculitis or kidney disease. Sixteen months later he developed rapidly progressive glomerulonephritis that responded to cyclophosphamide treatment. While undergoing treatment for GPA, he was found to have a lung mass identified as small cell lung cancer. This mass was present at the time of the IgG4-RD diagnosis. GPA can be confused with IgG4-RD histologically and they rarely coexist. ANCA antibodies are primarily IgG4 subclass. IgG4-RD has been associated with cancer and may improve prognosis. We speculate that this patient may have had small cell lung cancer that incited an IgG4 predominant immune response with coexistent ANCA antibodies that eventually resulted in GPA. Immunosuppressive treatment of GPA likely accelerated the progression of the lung cancer.


Assuntos
Granulomatose com Poliangiite/patologia , Doença Relacionada a Imunoglobulina G4/patologia , Neoplasias Pulmonares/patologia , Pulmão/patologia , Carcinoma de Pequenas Células do Pulmão/patologia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Evolução Fatal , Glomerulonefrite/complicações , Granulomatose com Poliangiite/complicações , Humanos , Imunoglobulina G/sangue , Doença Relacionada a Imunoglobulina G4/imunologia , Imunossupressores/uso terapêutico , Pulmão/diagnóstico por imagem , Pulmão/cirurgia , Neoplasias Pulmonares/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Carcinoma de Pequenas Células do Pulmão/complicações , Carcinoma de Pequenas Células do Pulmão/tratamento farmacológico , Cirurgia Torácica Vídeoassistida/métodos
13.
BMJ Case Rep ; 12(2)2019 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-30824462

RESUMO

A 62-year-old man presented with excruciating joint pains, back stiffness and numbness of his hands and feet. Over the past 18 months, he had experienced similar episodes for which the diagnoses of bilateral carpal tunnel syndrome and lateral epicondylitis had been made. Physical examination revealed polyarticular arthritis affecting the shoulders, wrists and right knee. Palpable purpura overlying the calves and ankles was present. Laboratory tests showed markedly elevated erythrocyte sedimentation rate and C-reactive protein in the setting of negative blood and urine cultures. Rheumatoid factor and antinuclear antibodies were negative. Chest CT demonstrated bilateral pulmonary infiltrates. A punch biopsy of the rash showed leukocytoclastic vasculitis. Anti-proteinase-3 titers returned strongly positive. A diagnosis of granulomatosis with polyangiitis was made. Treatment with high-dose steroids, followed by rituximab resulted in normalisation of inflammatory markers with subsequent resolution of joint pains, rash and pulmonary infiltrates and improvement of neuropathic symptoms.


Assuntos
Síndrome do Túnel Carpal , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/patologia , Cotovelo de Tenista , Anti-Inflamatórios/uso terapêutico , Artralgia/etiologia , Biópsia , Diagnóstico Diferencial , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Fatores Imunológicos/uso terapêutico , Pulmão/diagnóstico por imagem , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Rituximab/uso terapêutico , Tomografia Computadorizada por Raios X
15.
Rheumatol Int ; 39(5): 885-892, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30887162

RESUMO

Nasal biopsy is the essential method for differentiating and diagnosing granulomatosis with polyangiitis (GPA) in patients with chronic rhinosinusitis. Nevertheless, in the real clinical settings, there are several cases unable for nasal biopsy. Hence, in this study, we investigated initial clinical manifestations and laboratory factors which could be helpful for diagnosing GPA in cases unable for nasal biopsy performance. We retrospectively reviewed the medical records of 45 patients with GPA. Twenty-five patients exhibited chronic rhinosinusitis, among which 16 patients underwent nasal biopsy. We applied the 2007 European Medicines Agency algorithm for the classification of GPA, the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis and the 2017 American College of Rheumatology/European League Against Rheumatism provisional classification criteria for GPA to them for reclassifying GPA. Among six patients without granuloma on nasal biopsy, three patients with only antineutrophil cytoplasmic antibody (ANCA) and chronic rhinosinusitis could be classified as GPA due to proteinase 3 (PR3)-ANCA (or cytoplasmic (C)-ANCA) positivity. Among nine patients without nasal biopsy, three patients with only chronic rhinosinusitis could be classified as GPA due to GPA-specific lung lesions. When we excluded an item of granuloma in ten GPA patients with granuloma on nasal biopsy, four patients without ANCAs could be classified as GPA due to GPA-specific lung lesions and cartilaginous involvement. In conclusion, PR3-ANCA (or C-ANCA) positivity, GPA-specific lung lesions and cartilaginous involvement could help physicians in charge make a final diagnosis of GPA in cases unable for nasal biopsy.


Assuntos
Granulomatose com Poliangiite/diagnóstico , Nariz/patologia , Rinite/patologia , Sinusite/patologia , Adolescente , Adulto , Idoso , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Biópsia , Doenças das Cartilagens/etiologia , Criança , Feminino , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/imunologia , Granulomatose com Poliangiite/patologia , Humanos , Pneumopatias/etiologia , Masculino , Pessoa de Meia-Idade , Mieloblastina/imunologia , Estudos Retrospectivos , Rinite/etiologia , Sinusite/etiologia , Adulto Jovem
17.
Rev. chil. radiol ; 25(1): 26-34, mar. 2019. tab, ilus
Artigo em Espanhol | LILACS | ID: biblio-1003747

RESUMO

Resumen:La granulomatosis con poliangeítis (GPA) es una vasculitis sistémica de pequeño vaso, que afecta más frecuentemente el tracto respiratorio y el riñón. Sus criterios diagnósticos se basan en la clínica, exámenes de laboratorio, imágenes e histología. El 90% son ANCA (anticuerpos anticitoplasma de neutrófilos) positivos. La histología muestra inflamación granulomatosa, necrosis y vasculitis. Los exámenes de imagen son de vital importancia en su estudio inicial y seguimiento, correspondiendo principalmente a técnicas tomográficas. La tomografía Computada (TC) es el método de elección para la evaluación de vía aérea superior y pulmón, con alta sensibilidad en afectación de cavidades nasal/paranasales, árbol bronquial y pulmón. La Resonancia Magnética está indicada en compromiso del sistema nervioso central y corazón. El PET/CT presenta alta sensibilidad en enfermedad tóraco-abdominal, es de utilidad en detectar lesiones no visibles con otras técnicas, y en control de tratamiento. El compromiso renal, de alta ocurrencia en GPA, presenta escasa traducción en las imágenes y es frecuentemente indetectable con imágenes, aunque el PET/CT puede ser positivo en casos de glomerulonefritis acentuada. La radiología simple no debe ser utilizada en el estudio de GPA dado su bajo rendimiento diagnóstico. El tratamiento se basa en terapia corticoidea e inmunosupresora. Las recaídas son frecuentes, por lo que estos pacientes requieren seguimiento a largo plazo.


Abstract:Granulomatosis with polyangiitis (GPA) is a systemic type of vasculitis that affects small vessels, most commonly involving the respiratory tract and kidneys. Diagnosis is based on clinical criteria, laboratory tests, imaging and histology. Ninety percent are ANCA (anti-neutrophilic cytoplasmic antibodies) positive. Histology demonstrates granulomatous inflammation, necrosis and vasculitis. Imaging studies are vital for the initial work-up and follow-up. Computed Tomography (CT) is the method of choice for evaluation of the upper airway and lungs, because of its high sensitivity detecting anomalies of paranasal sinuses, bronchial tree and lungs. Magnetic Resonance is indicated for evaluation of the central nervous system and heart. PET/CT has high sensitivity for thoracic and abdominal disease, is useful at detecting lesions not seen with other imaging techniques, and for follow-up. Renal involvement, very frequent on GPA, is usually undetectable at imaging, but may be seen at PET/CT in cases of marked glomerulonephritis. Plain X-rays should not be used for evaluation of GPA because of their low diagnostic performance. Treatment is based on corticosteroid and immunosuppressive therapy. Relapses are frequent, so these patients require long-term follow-up.


Assuntos
Humanos , Tomografia Computadorizada por Raios X , Granulomatose com Poliangiite/diagnóstico por imagem , Tomografia por Emissão de Pósitrons , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/patologia , Nefropatias/etiologia , Pneumopatias/etiologia
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