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1.
Saudi Med J ; 43(1): 105-107, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35022291

RESUMO

This is a case report of a 31-year-old lady who is known to have ulcerative colitis for 15 years and was on mesalazine. She presented with periorbital swelling, sinusitis, epistaxis, and was found to have positive anti-neutrophil cytoplasmic antibody and anti-proteinase-3 of a high titer. Biopsy from the maxillary sinus showed chronic non-specific inflammation and biopsy from the periorbital fat revealed inflammation and granulomatous changes. She had no other organ involvement. She was diagnosed with a limited form of granumatosis and polyangiitis and treated with methotrexate and prednisolone. The symptoms disappeared after treatment.


Assuntos
Colite Ulcerativa , Granulomatose com Poliangiite , Adulto , Anticorpos Anticitoplasma de Neutrófilos , Biópsia , Colite Ulcerativa/tratamento farmacológico , Feminino , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Sulfassalazina
2.
Clin Nucl Med ; 47(1): 83-85, 2022 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-34319963

RESUMO

ABSTRACT: A 57-year-old woman was referred for progressive dyspnea. CT showed a tracheal mass, suspicious of tracheal neoplasm. The lesion was partially resected, and nonspecific granulation tissue was observed on histology. Her symptoms and CT findings worsened. FDG PET/CT showed increased FDG accumulation in the nasal septum and left eustachian tube in addition to the tracheobronchial lesions. Although the patient was ANCA (antineutrophil cytoplasmic antibodies) negative, a differential diagnosis of granulomatosis with polyangiitis was established and confirmed pathologically. FDG PET/CT was useful for diagnosis of ANCA-negative granulomatosis with polyangiitis, in which tracheobronchial and cartilage lesions were prominent.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite , Cartilagem , Feminino , Fluordesoxiglucose F18 , Granulomatose com Poliangiite/diagnóstico por imagem , Humanos , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada
3.
Curr Opin Rheumatol ; 34(1): 33-38, 2022 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-34738981

RESUMO

PURPOSE OF REVIEW: This review paper evaluates the use of patient reported outcome (PROs) in systemic vasculitis and the increasing incorporation of these measures in the evaluation of clinical outcomes and healthcare provision. RECENT FINDINGS: Generic PROs such as the SF-12, SF-36, EQ-5D have been used to evaluate health-related quality of life (HRQOL) across the spectrum of vasculitis; including giant cell arteritis, antineutrophil cytoplasmic antibody (ANCA)-related vasculitis and immunoglobulin A vasculitis (IgA) vasculitis. More recently disease-specific PROs have been developed including the associated vasculitis (AAV)-PRO and GCA-PRO, whilst further work is ongoing including a Steroid-PRO. SUMMARY: Generic and disease-specific PROs are complimentary in nature, but the advent of disease-specific PROs allows evaluation of the impact of specific symptoms and intervention on patient HRQOL. Following the COVID-19 pandemic, the advent of increasing virtual work has brought the potential for electronic-PRO measures to the forefront and is a current area of interest.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , COVID-19 , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Anticorpos Anticitoplasma de Neutrófilos , Humanos , Pandemias , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , SARS-CoV-2
4.
J Clin Rheumatol ; 28(1): 44-51, 2022 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-34941619

RESUMO

ABSTRACT: Most of the existing literature, including epidemiological studies and clinical trials, on antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) include North American (mainly United States and Canada), European, and Asian populations. Few studies have focused on multiethnic populations such as the one from Latin America. Racial and ethnic differences in the incidence of AAV could partially explain the comparatively low number of AAV studies originating in Latin America. However, given the racial/ethnic diversity as well as socioeconomic differences existing in this region, better reporting of AAV presentations and outcomes in Latin America could highlight valuable gaps on the understanding and treatment of these patients. Recently, larger case series and studies have provided better clinical information regarding AAV patients in Latin American countries; however, further information is needed to address gaps such as risk factors, genetic profiles, clinical features, and predictors of clinical outcomes. For these reasons, we have performed a systematic literature review to enhance our understanding of AAV patients in Latin America. We have included 11 articles focused on the epidemiological and clinical features of AAV in Latin America; some similarities and differences with AAV in other regions are shown in these articles. We have identified differences in their prevalence across Latin American countries, which may reflect reporting bias or true ethnic differences among the countries. Our findings should encourage further investigation into AAV in Latin America; such studies will hopefully lead to the optimal management of these patients.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/epidemiologia , Anticorpos Anticitoplasma de Neutrófilos , Humanos , Incidência , América Latina/epidemiologia , Fatores de Risco
5.
J Med Case Rep ; 15(1): 618, 2021 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-34963497

RESUMO

BACKGROUND: Dysthyroid optic neuropathy is the most commonly suspected diagnosis of optic neuropathy in Graves' patients; however, other causes need to be ruled out. We present a unique case of optic neuropathy secondary to hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be antithyroid drug related. CASE PRESENTATION: A 79-year-old Japanese male presented with acute visual loss in the left eye. He had a 24-year history of Graves' disease and was taking methimazole. Best-corrected visual acuity was 0.8 in the right eye and light perception in the left eye, and relative afferent pupillary defect in the left eye was seen. Ocular movement was normal, and there were no findings explaining visual loss in intermediate optic media and fundus in the left eye. Contrast-enhanced magnetic resonance imaging demonstrated thickened dura mater. Tests for myeloperoxidase-antineutrophil cytoplasmic antibody, proteinuria, and hematuria were positive; pulmonary nodule lesions and a blood clot in the left lower leg were also found. After excluding the presence of diseases that could lead to hypertrophic pachymeningitis, we diagnosed optic neuropathy due to hypertrophic pachymeningitis with granulomatosis with polyangiitis-a subtype of antineutrophil cytoplasmic antibody-associated vasculitis. Since he had history of using methimazole, antineutrophil cytoplasmic antibody-associated vasculitis was considered as drug related. We started high-dosage steroid pulse therapy followed by 1 mg/kg body weight daily of oral prednisolone, and subsequently tapered. Methimazole was stopped. Best-corrected visual acuity recovered to 0.9, 2 weeks after starting treatment. Though myeloperoxidase-antineutrophil cytoplasmic antibody remained negative, the symptom relapsed 6 months after treatment initiation. We gave a second high-dose steroid pulse therapy followed by prednisolone tapered together with methotrexate. Remission remained, and using 4 mg/week methotrexate without prednisolone, myeloperoxidase-antineutrophil cytoplasmic antibody was kept within the normal limit until now, 4 years after onset. CONCLUSION: We present a case of optic neuropathy with hypertrophic pachymeningitis related to antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be drug related. The patient had good visual recovery after quitting the drug and receiving immunosuppressive therapy with systemic steroids. Hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis related to antithyroid drugs should be considered as a differential diagnosis for optic neuropathy in Graves' patients in whom optic nerve compression is not obvious.


Assuntos
Granulomatose com Poliangiite , Doença de Graves , Meningite , Doenças do Nervo Óptico , Idoso , Anticorpos Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite/complicações , Humanos , Masculino , Nervo Óptico , Doenças do Nervo Óptico/diagnóstico , Doenças do Nervo Óptico/tratamento farmacológico , Doenças do Nervo Óptico/etiologia
6.
Medicina (Kaunas) ; 57(12)2021 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-34946270

RESUMO

Due to complex comorbidity, high infectious complication rates, an elevated risk of relapsing for primary renal disease, as well as inferior recipient and allograft survivals, individuals with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAVs) are often considered as poor transplant candidates. Although several aspects of recurrent and de novo AAVs remain unclear, recent evidence suggests that kidney transplantation (KT) represents the best option, which is also the case for this particular subgroup of patients. Special counselling and individualized approaches are strongly recommended at the time of enlistment and during the entire post-transplant follow-up. Current strategies include avoiding transplantation within one year of complete clinical remission and thoroughly assessing the recipient for early signs of renal or systemic vasculitis. The main clinical manifestations of allograft AAV are impaired kidney function, proteinuria, and hematuria with ANCA positivity in most cases. Mixed results have been obtained using high-dose steroids, mycophenolate mofetil, or cyclophosphamide. The aim of the present review was to summarize the available literature on AAVs in KT, particularly focusing on de novo pauci-immune glomerulonephritis.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Transplante de Rim , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Anticorpos Anticitoplasma de Neutrófilos , Humanos , Rim , Proteinúria
7.
Ann Palliat Med ; 10(10): 11209-11215, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34763478

RESUMO

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg-Strauss syndrome, is a rare chronic necrotizing eosinophilic granulomatous inflammatory disease characterized by eosinophil-rich granulomatous inflammation and small- to medium-size vessel vasculitis associated with bronchial asthma and eosinophilia, which is positive for anti-neutrophil cytoplasmic antibody (ANCA) in approximately 50-70% of cases. We report a case of a 23-year-old woman was admitted to our hospital because of a of small vesicles on both lower limbs and a 4-month history of small scattered skin rash with pruritus V6 on both lower limbs four-month history of scattered skin rash with pruritus. Laboratory data from peripheral blood revealed leukocytosis, eosinophilia, thrombocytosis, hyperfibrinolysis, and mild renal injury. Her ANCA was negative, and the skin pathological examination showed granuloma lesions with eosinophils, while elevated eosinophils were also found in the bone marrow. EGPA was diagnosed. On the other hand, the patient had 2-year-long rhinosinusitis, 9-month-long nephrotic syndrome, and 1-month-long dry cough, which might be a type of asthma. With steroid therapy followed by systemic immunomodulatory therapy, the patient's symptoms were relieved. Our case report and literature review highlight the importance of recognizing cough variant asthma as an initial presenting symptom of EGPA, providing an opportunity for early diagnosis and treatment to reduce the risk of further disease progression and morbidity.


Assuntos
Asma , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Anticorpos Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/diagnóstico , Tosse/etiologia , Feminino , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Humanos , Adulto Jovem
8.
Eur Respir Rev ; 30(162)2021 Dec 31.
Artigo em Inglês | MEDLINE | ID: mdl-34750115

RESUMO

Over the past three decades, an increasing number of publications have reported the association between interstitial lung disease (ILD) and anti-neutrophil cytoplasmic antibody (ANCA) or ANCA-associated vasculitis (AAV). With this increased awareness, we have reviewed the literature to date and provide an update in this narrative review. The vast majority of cases of ILD have been shown to be in the setting of positive anti-myeloperoxidase antibody and can be present in up to 45% of patients of microscopic polyangiitis, though cases of ILD associated with proteinase 3 ANCA have rarely been reported. Pulmonary fibrosis and ANCA positivity can occur with or without systemic involvement. The pathogenetic mechanisms establishing the relationship between ANCA and the development of pulmonary fibrosis remain unclear. Histologic and radiographic features of ANCA-ILD most commonly reveal usual interstitial pneumonia or non-specific interstitial pneumonia patterns, though other atypical features such as bronchiolitis have been described. ILD in the setting of AAV has been associated with worse outcomes, and thus early identification and treatment in these patients is appropriate. We advocate that ANCA antibody testing be performed as a baseline evaluation in patients presenting with idiopathic interstitial pneumonia. Suggested treatment of ANCA-ILD includes immunosuppression and/or antifibrotic agents, though supporting data and clinical trials to substantiate use of these therapies are needed.


Assuntos
Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Anticorpos Anticitoplasma de Neutrófilos , Humanos , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/terapia , Peroxidase
10.
BMJ Case Rep ; 14(10)2021 Oct 07.
Artigo em Inglês | MEDLINE | ID: mdl-34620637

RESUMO

We present a patient with systemic symptoms including 4 months of dyspnoea worsened with exertion, fatigue, rhinorrhoea, intermittent facial swelling, generalised lymphadenopathy and weight loss. Laboratory studies demonstrated proteinuria and eosinophilia. His serology was consistent with Epstein-Barr Virus (EBV) reactivation. A lymph node biopsy was consistent with EBV-associated reactive lymphoid hyperplasia. He was told to continue symptomatic treatment for EBV infection. After several admissions, vasculitis workup and myeloperoxidase-antineutrophil cytoplasmic autoantibody (ANCA) studies were positive. Evolution of clinical symptoms, laboratory parameters and our literature review suggested the diagnosis of EBV-associated ANCA vasculitis. Steroids were started after the patient continued to deteriorate; the viral load started increasing, so we added valganciclovir with favourable clinical response and no relapse during the follow-up for 6 months. This suggests that with evidence of viraemia (primary or reactivation), antiviral treatment likely has clinical benefit while immunosuppression is being considered.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Infecções por Vírus Epstein-Barr , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Anticorpos Anticitoplasma de Neutrófilos , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/diagnóstico , Infecções por Vírus Epstein-Barr/tratamento farmacológico , Herpesvirus Humano 4 , Humanos , Masculino , Peroxidase
11.
Front Immunol ; 12: 762006, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34659268

RESUMO

As the coronavirus disease 2019 (COVID-19) pandemic is ongoing and new variants of severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) are emerging, there is an urgent need for COVID-19 vaccines to control disease outbreaks by herd immunity. Surveillance of rare safety issues related to these vaccines is progressing, since more granular data emerge with regard to adverse events of COVID-19 vaccines during post-marketing surveillance. Interestingly, four cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) presenting with pauci-immune crescentic glomerulonephritis (GN) after COVID-19 mRNA vaccination have already been reported. We here expand our current knowledge of this rare but important association and report a case of AAV presenting with massive rhabdomyolysis and pauci-immune crescentic GN after Pfizer-BioNTech COVID-19 mRNA vaccination. As huge vaccination programs are ongoing worldwide, post-marketing surveillance systems must continue to assess vaccine safety important for the detection of any events associated with COVID-19 vaccination. This is especially relevant in complex diseases where diagnosis is often challenging, as in our patient with AAV presenting with massive rhabdomyolysis and pauci-immune crescentic GN.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/patologia , Vacinas contra COVID-19/efeitos adversos , Glomerulonefrite/patologia , Rabdomiólise/patologia , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/imunologia , Anticorpos Anticitoplasma de Neutrófilos/sangue , Anticorpos Anticitoplasma de Neutrófilos/imunologia , COVID-19/prevenção & controle , Vacinas contra COVID-19/imunologia , Feminino , Glomerulonefrite/diagnóstico , Glomerulonefrite/imunologia , Humanos , RNA Mensageiro/imunologia , Rabdomiólise/diagnóstico , Rabdomiólise/imunologia
12.
Yonsei Med J ; 62(11): 1016-1022, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34672135

RESUMO

PURPOSE: We investigated whether serum clusterin levels could reflect the current antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-specific indices. MATERIALS AND METHODS: Fifty-seven patients with AAV and 40 healthy controls were included in this study. AAV-specific indices included the Short-Form 36-Item Health Survey Physical and Mental Component Summaries (SF-36 PCS and MCS) scores, Birmingham vasculitis activity score (BVAS), five-factor score (FFS), and vasculitis damage index. Clinical and laboratory data and AAV-specific indices were obtained at blood collection. The highest tertile of BVAS (≥16) was defined as high activity of AAV. RESULTS: The median age of AAV patients was 64.0 years and 19 patients were male. SF-36 PCS score (r=0.328), SF-36 MCS score (r=0.289), BVAS (r=-0.404), erythrocyte sedimentation rate (r=-0.336), and C-reactive protein levels (r=-0.421) were significantly correlated with serum clusterin levels. In the multivariable linear regression analysis using AAV-specific indices and serum clusterin levels, both FFS (ß=0.412) and serum clusterin levels (ß=-0.250) were significantly associated with BVAS. When the optimal serum clusterin cut-off level for high activity of AAV was identified as 130.45 µg/mL, patients with serum clusterin level ≤130.45 µg/mL had a significantly higher risk for high activity of AAV than did those without (relative risk 7.194). Patients with AAV exhibited significantly lower serum clusterin levels than did healthy controls (168.2 µg/mL vs. 230.5 µg/mL). CONCLUSION: Serum clusterin levels could reflect the current disease activity in patients with AAV.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Anticorpos Anticitoplasma de Neutrófilos , Clusterina/sangue , Sedimentação Sanguínea , Humanos , Masculino , Pessoa de Meia-Idade
13.
BMJ Case Rep ; 14(10)2021 Oct 27.
Artigo em Inglês | MEDLINE | ID: mdl-34706910

RESUMO

Granulomatosis with polyangiitis (GPA) is a rare disorder characterised by inflammation of small-sized and medium-sized blood vessels that result in damage to various organ systems, but it most commonly affects the respiratory tract and kidneys. It is one of the few entities that can present with ocular inflammation as well as renal impairment at the same time. We describe a case of a 38-year-old man with conjunctivitis, episcleritis, anterior uveitis as a first manifestation of GPA. His presentation with red eye and anterior uveitis prompted further workup, which revealed acute renal failure (creatinine 442 mmol/L), elevated inflammatory markers (erythrocyte sedimentation rate of 85 mmol/hour and C reactive protein of 72 mg/L), and a c-antineutrophil cytoplasmic antibody titre >8. An urgent renal biopsy was performed demonstrating necrotising crescentic glomerulonephritis, which led to the final diagnosis of GPA. Treatment induction with intravenous methylprednisolone and plasmapheresis followed by an oral prednisone taper and intravenous rituximab infusions leading to resolution of all symptoms and normalisation of kidney function. This report highlights conditions that can present with both ocular inflammation and renal dysfunction with a focus on GPA and its ocular manifestations.


Assuntos
Conjuntivite , Granulomatose com Poliangiite , Esclerite , Uveíte Anterior , Adulto , Anticorpos Anticitoplasma de Neutrófilos , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Humanos , Masculino , Esclerite/diagnóstico , Esclerite/tratamento farmacológico , Esclerite/etiologia , Uveíte Anterior/diagnóstico , Uveíte Anterior/tratamento farmacológico , Uveíte Anterior/etiologia
16.
Iran J Kidney Dis ; 15(5): 391-394, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34582374

RESUMO

Small vessel necrotizing vasculitis is divided into two groups; Immune complex mediated and Pauci immune vasculitis. Hemolytic uremic syndrome (HUS) is a rare disease manifested as microangiopathic hemolytic anemia, thrombocytopenia and renal involvement. The coexistence of ANCA negative vasculitis and atypical HUS (aHUS) is rare. We describe a case of a 40 years old lady with rapidly declining kidney function. Renal Biopsy revealed Crescentic necrotizing glomerulonephritis (CGN). She was treated with plasmapheresis alternating with hemodialysis (HD) and immunosuppressive therapy. One month later she developed hemolytic anemia with peripheral schistocytes and thrombocytopenia and diagnosed as aHUS. Same treatment continued and her aHUS resolved spontaneously over one week. However her kidney functions didn't improve and ended up with end stage renal disease (ESRD). DOI: 10.52547/ijkd.6443.


Assuntos
Síndrome Hemolítico-Urêmica Atípica , Falência Renal Crônica , Vasculite , Adulto , Anticorpos Anticitoplasma de Neutrófilos , Síndrome Hemolítico-Urêmica Atípica/complicações , Síndrome Hemolítico-Urêmica Atípica/diagnóstico , Síndrome Hemolítico-Urêmica Atípica/terapia , Feminino , Humanos , Falência Renal Crônica/diagnóstico , Falência Renal Crônica/terapia , Diálise Renal
17.
Autoimmun Rev ; 20(11): 102940, 2021 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-34509652

RESUMO

OBJECTIVE: Salivary gland involvement in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is rare, but can lead to the misdiagnosis of other diseases. The objective of this study was to clarify the characteristics of patients with salivary gland involvement. METHODS: We conducted a systematic literature review of articles reporting salivary gland involvement in ANCA-associated vasculitis from the inception dates until May 2, 2021. RESULTS: We identified 58 patients with salivary gland involvement. The mean age was 52 years, and men were predominantly affected (59%). Half of the patients presented with fever. Swelling of the salivary gland was the initial manifestation in 88% of the patients, unilaterally affected in 53%, and painful in 47%. The affected salivary glands were as follows: parotid gland alone (53%), submandibular gland alone (33%), and both parotid and submandibular glands (14%). Additionally, two patients had sublingual gland involvement. The most frequent clinical diagnosis was granulomatosis with polyangiitis (83%), followed by eosinophilic granulomatosis with polyangiitis (17%), while no patient was diagnosed with microscopic polyangiitis. PR3-ANCA positivity (72%) was predominant to MPO-ANCA positivity (6%), and ANCA was negative in 22% of the patients. Among 37 ANCA-positive patients, 6 patients (16%) were initially ANCA-negative, but subsequently became positive during the clinical course. The serum C-reactive protein levels were elevated in all the examined patients. On contrast-enhanced computed tomography, a finding suggestive of necrosis, which was heterogeneous enhancement with low-density areas, was found in 33% of the patients. Vasculitis, granulomatous inflammation, necrosis, or the presence of multinucleated giant cells was found in 83% of the biopsy samples of the affected salivary gland. Glucocorticoids with or without other immunosuppressive agents, such as cyclophosphamide were effective in most patients, but twelve patients (21%) experienced a relapse of the disease and nine patients (16%) died during the clinical course. CONCLUSION: Salivary gland involvement can be an initial manifestation of ANCA-associated vasculitis. The recognition of this unusual atypical presentation is important for the early and accurate diagnosis and treatment.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos , Humanos , Masculino , Pessoa de Meia-Idade , Glândulas Salivares
18.
Medicina (Kaunas) ; 57(9)2021 Aug 24.
Artigo em Inglês | MEDLINE | ID: mdl-34577784

RESUMO

BACKGROUND AND OBJECTIVES: Cocaine users may present with positive antineutrophil cytoplasmic antibodies (ANCA) and severe midline destructive lesions (CIMDL) which are histologically characterized by massive apoptosis. However, histopathological and laboratory studies suggest that autoimmunity may not be the main pathogenic driver. We analyzed gene expression both in cell lines of nasal mucosa exposed to cocaine and in CIMDL patients to determine whether genetic predisposition might cause such lesions, which are observed in a minority of cocaine abusers. MATERIALS AND METHODS: The genetic expression profile of nasal mucosa exposed to cocaine was analyzed. Rare variants of expressed genes were searched in patients with CIMDL using exome sequencing and bio-informatics. RESULTS: We identified 462 genes that were induced by cocaine, mainly related to apoptosis and autophagy in response to oxidative stress. Under the hypothesis that genes linked to the phenotype are also induced by cocaine itself, a rare variants burden test was performed to select genes that were significantly enriched in rare mutations. Next, 11 cocaine abusers with CIMDL and no other relevant medical comorbidities underwent exome sequencing, and 12 genes that were significantly enriched in the burden test and present in at least 10 patients were identified. An in-depth analysis of these genes revealed their involvement in apoptosis, tissue homeostasis, autophagy, and response to oxidative stress. CONCLUSIONS: Oxidative stress and rare genetic alterations in the response to reactive oxygen species, apoptosis, autophagy, and tissue regeneration are plausible drivers of damage affecting nasal mucosa exposed to cocaine crystals and, consequently, the pathogenic mechanism behind CIMDL.


Assuntos
Transtornos Relacionados ao Uso de Cocaína , Cocaína , Anticorpos Anticitoplasma de Neutrófilos , Cocaína/efeitos adversos , Transtornos Relacionados ao Uso de Cocaína/genética , Expressão Gênica , Humanos
19.
Viruses ; 13(9)2021 08 29.
Artigo em Inglês | MEDLINE | ID: mdl-34578298

RESUMO

A relationship is emerging between SARS-CoV-2 infections and ANCA-associated vasculitis (AAV) because: (i) the pulmonary involvement of COVID-19 may mimic that observed in patients with AAV; (ii) the two diseases may occur together; (iii) COVID-19 may trigger AAV. However, few cases of AAV have been identified so far in COVID-19 patients. To define the frequency of ANCA autoimmunity in patients with SARS-CoV-2 infection, we analyzed the serum ANCAs and the serum PR3 and MPO antigens by immunoassays in 124 adult patients with a diagnosis of SARS-CoV-2 infection (16 were asymptomatic and 108 were hospitalized) and 48 control subjects. The serum ANCAs were significantly higher in the hospitalized patients compared with either the controls or the asymptomatic patients and increased with the progression of the COVID-19 severity. After one week of hospitalization, the values were significantly lower. In contrast, no differences emerged among the controls, asymptomatic and hospitalized patients for the PR3 and MPO serum levels. None of the patients had clinical signs of AAV with the exception of a severe pulmonary involvement. Further studies are necessary to define whether the increase in the serum ANCAs might mask subclinical vasculitis in a percentage of patients with SARS-CoV-2 infection or it is an epiphenomenon of SARS-CoV-2 infection with no clinical manifestations.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/sangue , COVID-19/sangue , COVID-19/virologia , SARS-CoV-2 , Adulto , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/sangue , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/etiologia , COVID-19/diagnóstico , COVID-19/imunologia , Suscetibilidade a Doenças , Feminino , Humanos , Imunoensaio , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Avaliação de Sintomas
20.
Pan Afr Med J ; 39: 162, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34539959

RESUMO

Neurofibromatosis type 1 is a common neurocutaneous syndrome, caused by an alteration of the NF-1 gene, which is a tumor suppressor. It has been reported to be associated with different types of benign and malignant tumors but its association to systemic diseases is uncommon and has not been reported previously to be associated with vasculitis particularly granulomatosis with polyangiitis (GPA). We report a case of a 17-year-old male patient, who, during his follow-up for neurofibromatosis type 1, in our outpatient consultation; we objectified hypereosinophilia at 1700 eosinophils/µl without a history of asthma or atopy. He reported a nasal obstruction with epistaxis and no rhinorrhea or pruritus. Physical examination revealed afebrile patient with the lesions of his neurofibromatosis type 1 without cutaneous rash or urticaria. Rhinoscopy didn't show any lesion. Laboratory tests revealed a normal renal function, negative 24-hour urine protein, and no biological inflammatory syndrome. Immunological tests noted positives cytoplasmic antineutrophil cytoplasmic antibodies, and a slight increase in antinuclear antibodies at 1/180. Extensive infectious research was negative. Computed tomography (CT) of the sinuses revealed a non-obstructive nasal septum deviation with anatomical variations, and a chest scan showed multiple bilateral pulmonary nodules and micronodules. After ruling out the other etiologies, we retained the diagnosis of granulomatosis with polyangiitis according to American College of Rheumatology (ACR) criteria 1990 and we could start early the treatment. To our knowledge, the association between Neurofibromatosis type 1 and vasculitis, particularly GPA, has not been reported previously, which makes our case original and confirms the utility of an extensive lesion assessment during the follow-up.


Assuntos
Granulomatose com Poliangiite/diagnóstico , Neurofibromatose 1/diagnóstico , Tomografia Computadorizada por Raios X , Adolescente , Anticorpos Anticitoplasma de Neutrófilos/imunologia , Granulomatose com Poliangiite/fisiopatologia , Humanos , Masculino , Neurofibromatose 1/fisiopatologia
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