Analysis of clinical features of 147 cases of ANCA-associated vasculitis / 中华风湿病学杂志

Objective:To analyze the clinical characteristics of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods:A retrospective study was conducted on AAV patients with long-term follow-up in the rheumatology outpatient clinic of Peking Union Medical College Hospital between February 2015 and February 2022. The demographic characteristics, clinical manifestations, concurrent events, treatment, and prognosis of the three clinical subtypes of AAV were collected and analyzed.Results:There were 71, 45, and 31 cases of granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatous polyangiitis (EGPA), respectively, among 147 patients. The ANCA positivity rates in the three groups were 91.5%, 95.6%, and 19.4% ( χ2=76.68, P<0.01), respectively. The upper respiratory tract and lungs were the most frequently affected organs in GPA and EGPA, and the kidneys and lower respiratory tract were the main organs involved in MPA. In addition, cardiac and neurological involvement and thrombosis rates were significantly higher in EGPA patients than in GPA and MPA (12.9%, 9.7%, 41.9% and 19.4%, respectively; χ2=8.51、7.13、7.54、0.02, P<0.05) .The median follow-up time for the three groups of patients was 43, 28, and 46 months respectively.Relapse was more common in patients with GPA and EGPA (up to 59.2% and 64.5%; χ2=11.26, P=0.004), with the lungs and ENT being the most common relapse organs (GPA of 61.9% and 40.5%, EGPA of 55.0% and 50.0%), the lungs and kidneys were the most common manifestations in MPA relapse (64.3% and 60.0%, respectively). The main therapeutic agents were glucocortoid (95.9%), cyclophosphamide (71.4%), methotrexate (54.4%), tripterygium wilfordiz (34.0%),mycophenolate mofetil (31.3%), azathioprine (29.3%), leflunomide (19.0%), rituximab (19.0%), and tacrolimus/cyclosporine (8.8%). There were 6 deaths (4.1%) occurred during the follow-up period of this study. Conclusion:The clinical features of AAV are similar to those reported in the literature and relapses are common>he vast majority of patients need to be treated with glucocorticoid combined with immunosuppressive agents.

The central nervous system involvement of patients with microscopic polyangiitis / 中华风湿病学杂志

Objective:To explore the clinical features and prognosis of central nervous system involvement in patients with microscopic polyangiitis (MPA).Methods:We retrospectively investigated the clinical data of 138 MPA patients hospitalized with MPA in Tianjin Medical University General Hospital from January 1, 2010 to November 1, 2019. Patients were divided into two groups according to whether they had the central nervous system (CNS) involvement or not and then Kaplan-Meier survival curve was used to analyze the survival rate between the two groups, Logistic regression model analysis was adopted to analyze risk factors, and P<0.05 was considered statistically significant. Results:①29 patients (21.0%)among the 138 MPA had CNS-affected, including 13(44.8%) males and 16(55.2%) females. CNS involvement was present at the diagnosis of MPA in 20 cases (69.0%) and after the diagnosis of MPA in 9 cases (31.0%). ②The clinical manifestations were motor impairment in 14 cases (48.3%), sensory impairment in 10 cases (34.5%), speech loss in 9 cases (31.0%), headache in 8 cases (27.6%), consciousness disorder in 7 cases (24.1%), dysphagia and bucking in 4 cases (13.8%), cranial nerves involvement in 3 cases (10.3%). The imaging manifestations of the head included infarction, hemorrhage, infarction with hemorrhage and linear dural thickening. Five patients received lumbar puncture. One patient showed elevation of cerebrospinal fluid pressure, 1 patient had elevated protein and 5 patients showed elevation of LDH.③Eighteen patients received glucocortoid combined with cyclophosphamide. CNS symptoms recurred in 6 patients, four patients had recurrent cerebral infarction. ④Median survival time was 55 months in the CNS affected group [95% CI=(14.215, 95.785)] and 86 months in the N-CNS group [95% CI=(24.378, 147.622)]. Kaplan-Meier survival curve showed that there was no significant difference in survival rate between the two groups ( χ2=0.07, P=0.794) . Conclusion:The central nervous system involvement of microscopic polyangiitis is not uncommon. The clinical manifestations are various, with motor impairment the most. The most common imaging manifestation is cerebral infarction and the patients mainly presenteas multiple cerebral infarction. However, the CNS involvement of microscopic polyangiitis is not associated with mortality.

Compromiso pulmonar en vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos / Pulmonary manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis

This article reviews the pulmonary manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis (AAV). Its frequency in the different phenotypes of the disease, clinical manifestations and updated therapeutic recommendations are reviewed, aiming to alert the medical community about the existence of these diseases. We pretend to stimulate a timely suspicion, diagnostic precision, and the implementation of effective therapies, to reduce the eventual sequelae derived from a diagnostic omission or an inappropriate treatment for the different clinical scenarios in which these diseases appear.

Peripheral ulcerative keratitis as presenting manifestation of systemic microscopic polyangiitis: a case report / Ceratite ulcerativa periférica como manifestação de poliangiíte microscópica sistêmica: relato de caso

ABSTRACT Microscopic polyangiitis is a rare autoimmune disease of unknown etiology, characterized by inflammation and necrosis of blood vessels. It forms a part of the antineutrophil cytoplasmic antibody-associated vasculitides-a heterogeneous group of disorders characterized by vasculitis. It is a systemic disease affecting multiple organs. The patients may present with a wide variety of symptoms. Ocular manifestations may present as its initial clinical symptoms, necessitating a multidisciplinary approach for reducing the morbidity and mortality. Early diagnosis aids in the formulation of appropriate treatment and prevention of further complications. Aggressive treatment, including surgery, is often necessary to limit structural damage and preserve visual function. We present the case of an 82-year-old woman who initially presented with peripheral ulcerative keratitis that led to the diagnosis of microscopic polyangiitis.

RESUMO A poliangeíte microscópica é uma doença autoimune rara de etiologia desconhecida, caracterizada por inflamação e necrose dos vasos sanguíneos. Faz parte das vasculites associadas a anticorpos citoplasmáticos antineutrófilos - um grupo heterogêneo de doenças caracterizadas por vasculite. É uma doença sistêmica que afeta vários órgãos. Os pacientes podem apresentar uma grande variedade de sintomas. As manifestações oculares podem apresentar-se como seus sintomas clínicos iniciais, necessitando de abordagem multidisciplinar para redução da morbimortalidade. O diagnóstico precoce ajuda na formulação do tratamento adequado e na prevenção de complicações futuras. O tratamento agressivo, incluindo cirurgia, muitas vezes é necessário para limitar o dano estrutural e preservar a função visual. Apresentamos o caso de uma mulher de 82 anos que inicialmente apresentou ceratite ulcerativa periférica que levou ao diagnóstico de poliangite microscópica.

Efficacy of rituximab in maintenance therapy for antineutrophil cytoplasmic antibody-associated vas-culitis / 中华风湿病学杂志

Objective:To evaluate the efficacy and safety of rituximab(RTX) as remission-mainten-ance therapy in antineutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV).Methods:Patients with AAV, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), treated with rituximab (RTX) in Peking Union Medical College Hospital during September 2005 to June 2021 were included into this study. Clinical data, relapse rate, time of first relapse and adverse events were collected and analyzed. The cumulative relapse rate was calculated by Kaplan-Meier, t test, and Man-Whithey U test and chi-square were used to compare differences between two groups. Results:① Thirty-nine AAV patients were enrolled, including 36 GPA and 3 MPA. During the 20(3, 104) months follow-up, 59.0%(23/39) patients had suffered relapses. The time for first relapse was 11(3, 42) months after remission. ② There were no difference in the relapse rate [60.0%(18/30) vs 55.6%(5/9), χ2=0.06, P=1.000), the time of first relapse [15(3, 42) vs 10(9, 30), Z=0.45, P=0.678], CD19 + B [23.5 (5, 148) cell/μl vs 3(2, 15) cell/μl, Z=0.57, P=0.605] and serum IgG [7.09(5.13, 13.90) g/L vs 9.72(5.32, 12.0) g/L, Z=0.36, P=0.770] between standard dose and low-dose groups. The rate of major relapse-free was significantly less in patients treated with standard dose than patients with reduced dose of RTX {87.1%[95% CI(73.4%, 100.8R%)] vs 64.3%[95% CI(23.1%, 105.4%)], χ2=7.59, P=0.006}. ③ There were no difference in relapse rate [50.0%(3/6) vs 60.6%(20/33), χ2=0.24, P=0.674], time of first relapse [23(6, 25) vs 11(3, 42), Z=0.05, P=0.982], CD19 + B[35(15, 50) cell/μl vs 10(0, 148) cell/μl, Z=0.95, P=0.382] and serum IgG[6.70(5.91, 7.49) g/L vs 7.69(3.78, 13.90) g/L, Z=0.48, P=0.700] between the fixed interval dosage and the on-demand dosage groups. There was no difference in the rate of major relapse-free between the two groups (100% vs 77.8%, χ2=1.79, P=0.181). ④ The incidence of infusion reaction was 5.1%(2/39) and infection was 20.5%(8/39). Serum IgG level was 4.37(3.78, 13.4) g/L at infection. There was no difference in safety between the standard and low-dose groups or between fixed interval and on-demand dosage groups ( P>0.05). Conclusion:There is no significant difference in relapse rate bet-ween the standard RTX dose and low-dose RTX induction therapy group, but the major relapse rate is sign-ificantly reduced in the standard dose RTX therapy. The relapse rate of fixed intervals dosage group is similar to that of on-demand dosage group. The safety profile of the standard dose and low-dose induction therapy groups or fixed intervals and on-demand dosage groups is similiar.

Characteristics and Prognosis of Microscopic Polyangiitis Patients with Diffuse Alveolar Hemorrhage and Interstitial Lung Disease / 中国医学科学杂志(英文版)

Objective To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage (DAH) and/or interstitial lung disease (ILD) secondary to microscopic polyangiitis (MPA) in a Chinese general hospital. Methods We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012. The patients were divided into the ILD, DAH, DAH combined with ILD (DAHILD), and no pulmonary involvement (NPI) groups according to pulmonary involvement patterns. The clinical characteristics at diagnosis were analyzed. The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.Results Of 193 newly diagnosed MPA patients, 181 patients were enrolled in the research, of which 19 had DAH alone, 96 had ILD alone, 18 had DAH and DAH concurrently, and 48 had NPI. The median of serum creatine level in the DAH group was 449 μmol/L, significantly higher than that in the ILD group (123 μmol/L, Nemenyi = -35.215, P = 0.045) and DAHILD group (359 μmol/L, Nemenyi = -43.609, P = 0.007). The median follow-up time was 67 (range: 1-199) months. Patients in the ILD group were older than those in the DAH group (median: 69 years vs. 57 years, Nemenyi = 43.853, P= 0.005). The patients with both DAH and ILD had combined features of the two subtypes, and the highest mortality (72.2% at the end of follow-up). The elevated white blood cell count was a risk factor for short-term death (OR = 1.103, 95%CI: 1.008-1.207, P = 0.032 for one month; OR = 1.103, 95%CI: 1.026-1.186, P = 0.008 for one year). Old age (HR= 1.044, 95%CI: 1.023-1.066, P < 0.001), cardiovascular system involvement (HR = 2.093, 95%CI: 1.195-3.665, P = 0.010), poor renal function (HR = 1.001, 95%CI: 1.000-1.002, P = 0.032) were risk factors for long-term death. Pulmonary infections (38/54) were the leading causes of death, especially for the patients with ILD. Besides, 49 patients suffered from pulmonary infections in the first year after diagnosis. Conclusions MPA patients who presented with different pulmonary involvement patterns have completely different clinical features. These subtypes probably have different pathogenesis and should be studied separately.

Analysis of clinical, pathological features and prognosis of microscopic polyangiitis / 中国医师进修杂志

Objectives:To analyze the clinical features of microscopic polyangiitis (MPA), and observe the clinical outcomes of different pathological types.Methods:The clinical data of 61 patients with MPA in Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine from January 2010 to December 2019 were analyzed retrospectively. According to age, the patients were divided into ≥ 60 years old group (46 cases) and<60 years old group (15 cases). According to the initial serum creatinine, the patients were divided into ≥ 500 μmol/L group (18 cases) and<500 μmol/L group (43 cases). The basic data and laboratory examination results of the patients were recorded, and the disease activity was evaluated by Birmingham systemic vasculitis activity score (BVAS). Twenty-three patients with complete pathological data were pathologically classified and followed up to assess their clinical outcomes. The progression to end-stage renal disease (ESRD) or death was defined as the endpoint.Results:Ferritin in ≥60 years old group was significantly higher than that in<60 years old group: 452 (289, 792) μg/L vs. 210 (119, 451) μg/L, and there was statistical difference ( P<0.05). The fever rate, hemoglobin and platelets in creatinine ≥ 500 μmol/L group were significantly lower than those in creatinine<500 μmol/L group: 3/18 vs. 48.8% (21/43), 77.5 (62.8, 86.0) g/L vs. 85.0 (77.0, 104.0) g/L and 192 (147, 234) × 10 9/L vs. 257 (208, 365) × 10 9/L, the gastrointestinal involvement and BVAS in creatinine ≥ 500 μmol/L group were significantly higher than those in creatinine<500 μmol/L group: 16/18 vs. 25.6% (11/43) and 20.0 (16.0, 23.3) scores vs. 15.0 (12.0, 19.0) scores, and there were statistical differences ( P<0.05 or<0.01). Pearson correlation analysis result showed that BVAS was positively correlated with creatinine ( r = 0.42, P<0.01), negatively correlated with hemoglobin ( r = -0.42, P<0.01), but it had no correlation with erythrocyte sedimentation rate and platelets ( r = 0.05 and 0.04, P>0.05). Among the 23 patients with completed the clinical outcome statistics, endpoint events occurred in 5 of 6 patients with crescent renal pathology, and in 7 of 12 patients with severe renal interstitial injury. Kaplan-Meier survival curve analysis result showed that the average survival time in ESRD MPA patients was significantly shorter than that in non ESRD MPA patients (41.2 months vs. 63.5 months), and there was statistical difference ( χ2 = 0.48, P = 0.028). Conclusions:The clinical manifestations of elderly MPA patients are similar to those of young MPA patients. Creatinine≥500 μmol/L or anemia at initial onset indicate higher vasculitis activity in MPA. The prognosis of MPA patients with pathological manifestations of crescent or severe interstitial injury is poor, and the survival rate of ESRD is lower than that of non ESRD patients.

Clinical characteristics and prognostic factors in an argentinian cohort with ANCA-associated vasculitis / Características clínicas y factores pronósticos en una cohorte argentina de vasculitis asociada a ANCA

Abstract ANCA-associated vasculitis is a heterogeneous group of rare autoimmune conditions of unknown cause. Clinical characteristics and prognostic factors were analyzed in 47 patients: 20 (42.5%) with granulomatosis with polyangiitis, 17 (36.2%) with microscopic polyangiitis, 6 (12.8%) with renal-limited vasculitis, and 4 (8.5%) with eosinophilic granulomatosis with polyangiitis. Mean age at diagnosis was 53.5 ± 16.5 years and the median of BVAS (Birmingham Vasculitis Activity Score) was 14 (4-42). The most frequent clinical manifesta tions were: general in 44 (93.6%), renal in 30 (63.8%) and respiratory in 28 (59.6%). All received corticosteroids at the beginning of treatment. Intravenous cyclophosphamide was associated in 20 (42.5%) and oral route in 14 (29.8%); azathioprine in 3 (6.4%) and rituximab in 2 (4.2%). At a median follow-up of 35.5 months (range 0.14- 234), 21 relapses were recorded in 14 patients. Overall mortality was 3.5 deaths per 100 patient-year in the whole group. Those over 55 years old, the presence of alveolar hemorrhage, those with FFS (Five Factor Score) of 2, and patients with MPA had poor prognosis. Renal involvement, ANCA pattern and BVAS were not associated to a poorer prognosis.

Resumen Las vasculitis asociadas a ANCA son un grupo heterogéneo de entidades autoinmunes, poco frecuentes, de etiología desconocida. Analizamos las características clínicas y factores pronóstico en 47 pacientes: 20 (42.5%) granulomatosis con poliangeítis, 17 (36.2%) poliangeítis microscópica, 6 (12.8%) vasculitis limitada al riñón y 4 (8.5%) granulomatosis eosinofílica con poliangeítis. La edad promedio al diagnóstico fue 53.5 ± 16.5 años y la mediana de BVAS (Birmingham Vasculitis Activity Score) 14 (4-42). Las manifestaciones clínicas más frecuentes fueron: generales en 44 (93.6%), renales 30 (63.8%) y respiratorias en 28 (59.6%). Todos recibieron corticoides al inicio del tratamiento. Se asoció ciclofosfamida endovenosa en 20 (42.5%) y oral en 14 (29.8%); azatioprina en 3 (6.4%) y rituximab en 2 (4.2%). En una mediana de seguimiento de 35.5 meses (rango 0.14-234), se registraron 21 recaídas en 14 pacientes. La mortalidad fue 3.5 por cien pacientes-año en todo el grupo. Los mayores de 55 años, con presencia de hemorragia alveolar, FFS (Five Factor Score) de 2, y los casos con poliangeítis microscópica tuvieron peor pronóstico. El compromiso renal, el patrón de ANCA y el BVAS no se asociaron a peor pronóstico.

Psicosis y alteraciones en la sensopercepción como manifestaciones atípicas de la poliangitis microscópica / Psychosis and alterations in sensory perception as atypical manifestations of microscopic polyangiitis

RESUMEN La poliangitis microscópica es una vasculitis sistémica, asociada a la positividad de anticuerpos anticitoplasma de neutrófilos, caracterizada por el compromiso necrosante de los vasos de pequeño calibre. Las manifestaciones clínicas de la poliangitis microscópica son variadas y las formas más severas se manifiestan con glomerulonefritis rápidamente progresiva y capilaritis pulmonar. El compromiso nervioso afecta principalmente el sistema periférico. La afectación a nivel central no es común, y hallazgos clínicos de psicosis y alteración del comportamiento son bastante infrecuentes en el contexto de estas enfermedades autoinmunes.

A B S T R A C T Microscopic polyangiitis is a systemic anti-neutrophil cytoplasmic antibody-associated vasculitis, and is associated with the necrotising small calibre vessels. Its clinical manifestations are varied, and the most severe forms manifest with rapidly progressive glomerulonephritis and pulmonary capillaritis. In the nervous system, it mainly involves the peripheral system. Involvement of the central nervous system is not common, and clinical findings of psychosis and behaviour alterations are infrequent.

Neuropatía periférica como manifestación unica de poliangeítis microscópica en la altura. Reporte de caso / Peripheral neuropathy as a unique manifestation of microscopic polyangetis in high altitude. Case report

Resumen La poliangeítis microscópica, es una vasculitis de vasos pequeños cuya incidencia es de 4 por 1 000 000 de habitantes y que frecuentemente se presenta asociada a manifestaciones de glomerulonefrítis rápidamente progresiva y capilaritis pulmonar. Las manifestaciones neurológicas son menos frecuentes. El diagnostico se confirma con serología positiva para Anticuerpos Anticitoplasma de neutrófilos (ANCA) y especificidad para patrón de fluorescencia perinuclear (P-ANCA) y Mieloperoxidasa (MPO). El tratamiento idóneo son los glucocorticoides e inmunosupresores. Se presenta el caso de un paciente de 69 años con poliangeitis microscópica que debuta con neuropatía periférica como única manifestación clínica, presentación que se considera atípica. Se plantea que ante la presencia de una neuropatía periférica aislada, deben efectuarse estudios para confirmar o descartar la presencia de Poliangeitis Microscópica en base a la gran variabilidad de su sintomatología clínica y potenciales formas de tratamiento.

Summary Microscopic polyangiitis is a small vessel vasculitis whose incidence is 4 per 1 000 000 inhabitants, frequently associated with manifestations of rapidly progressive glomerulonephritis and pulmonary capilaritis, and a less frequent neurological involvement. The diagnosis is confirmed with a positive serology for antineutrophil cytoplasmic antibodies (ANCA) and specificity for fluorescence pattern perinuclear (P-ANCA) and myeloperoxidase (MPO). The ideal treatment is a combination of glucocorticoids and immunosuppressants. The case is presented of a 69-year-old patient with microscopic polyangiitis who debuts with peripheral neuropathy as the only manifestation, an atypical feature. The suggestion is made that in the presence of an isolated peripheral neuropathy, studies should be done to rule out the presence of Microscopic Polyangiitis, given its great variability in clinical presentation and potential treatments.

Vasculitis asociada a anticuerpos anticitoplasma de neutrófilos: poliangeítis microscópica / Antineutrophil Cytoplasmic Antibody Associated Vasculitis: Microscopic Polyangiitis

La poliangeítis microscópica se define como una enfermedad autoinmune, multisistémica, de rara incidencia, asociada a anticuerpos anticitoplasma de neutrófilos. Se caracteriza por presentar vasculitis necrotizante de pequeños vasos, no granulomatosa y ausencia de depósito de inmunocomplejos, que afecta predominantemente el tejido renal, pulmonar y cutáneo. Se presenta un paciente masculino, 49 años, sin antecedentes patológicos. Presenta cuadro clínico de 30 días de evolución con tos seca de tipo irritativa, que evoluciona a tos productiva, hemoptisis, disnea de moderado esfuerzo, astenia y edema con fóvea en zonas en declive. Tenía anemia grave y los estudios imagenológicos de tórax evidenciaron infiltrados difusos con aspecto de vidrio deslustrado. Se inició terapia inmunosupresora con metilprednisolona y ciclofosfamida y profilaxis antibiótica. Se obtuvo una disminución en el puntaje de la escala de actividad vasculítica y la posterior remisión; sin embargo, debido al daño tisular renal, no hubo mejoría en la tasa de filtrado glomerular, por lo que el paciente se mantiene en terapia de sustitución renal permanente. Es trascendental el conocimiento de las manifestaciones clínicas, procedimiento diagnóstico y tratamiento de esta enfermedad, ya que, a pesar de su rara incidencia, posee una llamativa morbimortalidad que puede modificarse significativamente con un diagnóstico y tratamiento oportunos(AU)

Microscopic Polyangiitis is defined as an autoimmune, multisystemic, low prevalence disease, associated to antineutrophil cytoplasmic antibody. Characterized by necrotizing vasculitis of small vessels, with no granulomatous inflammation and absence of immune complex deposits, predominantly affecting renal, pulmonary and cutaneous tissue. 49-year old male patient with no past medical history. Refers symptomatology that started thirty days ago, characterized by dry cough, that evolves to productive cough, hemoptysis, exertional dyspnea, asthenia and pitting edema. Laboratory tests show severe anemia, glomerular filtration rate of 6.9 ml/min/1.73m2, hematuria, proteinuria, positive presence of antineutrophil cytoplasmic antibodies and anti-myeloperoxidase antibodies, additional thoracic imaging shows ground glass opacities consolidations. Immunosuppressive therapy is started with Methylprednisolone and Cyclophosphamide along with antibiotic prophylaxis, resulting in a decrease in the vasculitis activity score with subsequent remission; however due to renal tissue damage, there is no improvement in the glomerular filtration rate and the patient continues receiving renal replacement therapy. Due to its striking morbidity and mortality, we consider of great importance the knowledge of its clinical presentation, diagnostic procedures and treatment, in order to obtain a positive impact on the patient's quality of life and survival rate(AU)

Paquimeningitis hipertrófica en vasculitis anca positivo: reporte de caso y revisión de la literatura / Hypertrophic pachymeningitis in positive ANCA vasculitis: case report and literature review

La paquimeningitis hipertrófica (PH), es una manifestación poco frecuente de la vasculitis asociada a anticuerpos anti-citoplasma de neutrófilo (ANCA). La literatura describe compromiso de sistema nervioso central (SNC) en 2-8% de los casos en pacientes con vasculitis pauciinmune. Se presenta el caso de un paciente con antecedente de vasculitis anti-mieloperoxidasa (MPO) con un mes de evolución de cefalea hemicraneana izquierda. La resonancia magnética cerebral contrastada evidencia marcado engrosamiento y realce meníngeo dural en el hemicráneo izquierdo, predominante en el tentorio y la fosa posterior. Se descartaron causas infecciosas por lo que se llegó a la conclusión de compromiso meníngeo asociado a vasculitis. Se inició manejo inmunosupresor con mejoría del cuadro clínico. La rápida identificación y manejo de esta entidad puede cambiar su pronóstico sombrío. Se realizó una revisión de la literatura para brindar una herramienta para la toma de decisiones para los médicos que se enfrentan a esta entidad.

Hypertrophic pachymeningitis (PH) is a rare manifestation of vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA). The literature describes central nervous system (CNS) involvement in 2-8% of cases in patients with pauciimmune vasculitis. We present the case of a patient with a history of anti-Myeloperoxidase (MPO) vasculitis with a 1-month history of left-sided headache. Contrast brain magnetic resonance was performed with evidence of marked thickening and dural meningeal enhancement in the left hemicranium, predominantly in the region of the tentorium and posterior fossa. Infectious causes were ruled out and the meningeal compromise associated with vasculitis was concluded. Immunosuppressive management was started with improvement of the clinical picture. Rapid identification and management of this entity can change its bleak outlook. A systematic review of the literature was carried out in order to provide a decision-making tool for physicians facing this entity.

Clinical characteristics and prognosis of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis / 第二军医大学学报

Objective: To analyze the clinical characteristics and prognosis of patients with anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV). Methods: Clinical data (general information, clinical manifestation, and laboratory indexes) of 168 patients newly diagnosed as AAV between Jul. 2008 and Dec. 2016 were retrospectively analyzed. Patients were followed up for a median period of 12 months (range, 1-96 months). End-point event was death. Results: There were 152 cases of microscopic polyangiitis (MPA), 14 cases of granulomatous polyangiitis (GPA) and 2 cases of eosinophilic granulomatosis with polyangiitis (EGPA). MPO-ANCA was positive in 145 (86.3%) patients and PR3-ANCA was positive in 20 (11.9%) patients. Forty-nine patients died, 91 patients survived, and 28 patients lost their visits. Interstitial lung disease and renal involvement were more common in MPA patients as compared with GPA patients (50.7% [77/152] vs 14.3% [2/14], 78.9% [120/152] vs 50.0% [7/14], all P<0.05). Estimated glomerular filtration rate (eGFR) in MPA patients was significantly lower than that in GPA patients (14.23 [7.27, 71.49] mL/[min · 1.73 m2] vs 104.08 [16.61, 135.72] mL/[min · 1.73 m2], P<0.05). Compared with PR3-ANCA positive patients, MPO-ANCA positive patients was more elder ([64.01 ± 10.62] years vs [50.50±16.88] years), had more renal involvement (77.9% [113/145] vs 50.0% [10/20]) and lower eGFR (19.00 [9.40, 42.85] mL/[min · 1.73 m2] vs 149.40 [86.75, 249.45] mL/[min · 1.73 m2], all P<0.05). Compared with the survivals, the dead patients were older ([67.45±10.61] years vs [61.98 ± 12.52] years), had higher incidence of interstitial lung disease (59.2% [29/49] vs 41.8% [38/91]), higher Birmingham vasculitis activity score (BVAS) (18.53 ± 8.02 vs 13.68 ± 5.98), lower eGFR (8.58 [5.73, 22.07] mL/[min · 1.73 m2] vs 45.15 [11.54, 120.79] mL/[min · 1.73 m2], lower blood sodium concentration (137.00 [134.00, 140.00] mmol/L vs 139.00 [136.00, 141.00] mmol/L), lower albumin level ([28.41 ± 5.24] g/L vs [31.92 ± 5.91] g/L), and higher serum D-dimer level (2.84 [1.20, 6.28] mg/L vs 2.24 [0.80, 3.69] mg/L) (all P<0.05). Multivariate Cox proportional regression analysis showed that age, eGFR, serum albumin level and BVAS were independent influence factors of death (hazard ratio =1.058, 0.987, 0.932, and 1.086, all P<0.05). Conclusion: The clinical manifestations of AAV are mostly pulmonary and renal involvement. Age, eGFR, serum albumin level and BVAS are independent risk factors of death in AAV patients. Interstitial lung disease, high level of D-dimer and hyponatremia may be associated with prognosis of AAV.

Microscopic polyangiitis and antiphospholipid syndrome. An uncommon association / Poliangitis microscópica y síndrome antifosfolípido. Una asociación infrecuente

ABSTRACT Antiphospholipid syndrome is frequently associated with systemic lupus erythematosus and other autoimmune diseases. However, coexistence with primary vasculitis has been poorly reported. The case is presented of a 67-year-old patient with a history of recurrent abortion and chronic pulmonary thromboembolism who was admitted due to haemopty sis. At the initial evaluation, a massive alveolar haemorrhage and glomerulonephritis were diagnosed. The results of the antibodies were positive for ANCA with P-type pattern, anti-myeloperoxidase antibodies, and antiphospholipid antibodies (anti-β2 IgG glycoprotein 1 and lupus anticoagulant). Diagnosis of ANCA positive vasculitis-type microscopic polyangiitis was made in association with antiphospholipid syndrome. Given the clinical context, it was decided to initiate intravenous methylprednisolone in pulses for 3 consecutive days, fol lowed by oral prednisone, and as maintenance therapy, rituximab and anticoagulation with warfarin were instituted. The clinical evolution of the patient was satisfactory, with symp tom control being achieved, as well as a significant improvement of renal and pulmonary function, with a decrease in the Birmingham vasculitis activity score (BVAS).

RESUMEN El síndrome antifosfolípido se asocia frecuentemente con lupus eritematoso sistémico y otras enfermedades autoinmunes. Sin embargo, la coexistencia con vasculitis primaria ha sido poco reportada. Se presenta el caso de una paciente de 67 arios de edad con historia de aborto recurrente y tromboembolismo pulmonar crónico, quien es admitida para estudio de hemoptisis. A la evaluación inicial se diagnosticó una hemorragia alveolar masiva y glomerulonefritis. El resultado de los anticuerpos fue positivo para anticuerpos anticitoplasma de neutrófilos (ANCA) con patrón tipo perinuclear, anticuerpos anti-mieloperoxidasa y anticuerpos antifosfolípidos (anti (32 glicoproteína 1 IgG y anticoagulante lúpico), configurándose el diagnóstico de vasculitis asociada a ANCA de tipo poliangitis microscópica en asociación con síndrome antifosfolípido. Dado el contexto clínico, se decidió iniciar metilprednisolona intravenosa en pulsos por 3 días consecutivos, seguida de prednisona oral, y como terapia de mantenimiento se instauró rituximab y anticoagulación con warfarina. La evolución clínica de la paciente fue satisfactoria, alcanzando control de síntomas e importante mejoría de la función renal y pulmonar, con disminución del score BVAS.

Vasculitis en adultos mayores. A propósito de dos casos y revisión de la literatura / Vasculitis in elderly. About two clinical cases and literature review

Resumen Actualmente vivimos un proceso de transición en la pirámide poblacional. Por lo tanto, se hacen más frecuentes las enfermedades renales en los ancianos. Así, las vasculitis primarias en adultos mayores son enfermedades raras, causadas por la inflamación de los vasos sanguíneos y muy poco diagnosticadas. La literatura se limita a escasos casos clínicos. La vasculitis por anticuerpo citoplasmático anti neutrófilo (ANCA) positivo (VAA) es más frecuente en personas mayores de 50 años. La detección de ANCA es de peor pronóstico en esta población. También, es importante la alta sospecha y diagnóstico oportuno, ya que así se puede brindar un tratamiento óptimo y disminuir las complicaciones propias de la enfermedad y aquellas asociadas a la inmunosupresión. Los adultos mayores con VAA tienen mayor compromiso renal, hipertensión, dislipidemia y mortalidad incrementada. Por lo que a continuación se detallan dos casos clínicos desde la fecha del diagnóstico, edad, valores de laboratorio de la presentación inicial y la escala de actividad de vasculitis de Birmingham confirmados con biopsia renal (ANCA positivo) identificados en un hospital de especialidades de Ecuador.

Abstract Currently we live a process of transition in the population pyramid. Therefore, kidney diseases become more frequent in the elderly. Thus, primary vasculitis in older adults are rare diseases, caused by the inflammation of blood vessels and poorly diagnosed. The literature is limited to few clinical cases. Cytoplasmic anti-neutrophil antibody (ANCA) positive vasculitis (AAV) is more common in people older than 50 years. The detection of ANCA gives a very poor prognosis in this population. Also, high suspicion and timely diagnosis is important, as this can provide optimal treatment and reduce the complications of the disease and those associated with immunosuppression. Older adults with AAV have greater renal involvement, hypertension, dyslipidemia and increased mortality. Therefore, two clinical cases from the date of diagnosis, age, laboratory values of the initial presentation and the scale of activity of Birmingham vasculitis confirmed with renal biopsy (ANCA positive) identified in a specialty hospital of Ecuador are detailed below.

Microscopic polyangiitis associated with subarachnoid hemorrhage / Journal of Rural Medicine

Microscopic polyangiitis (MPA), an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a systemic disease that damages all organs through predominantly affecting small vessels. However, few cases of MPA are related to aneurysms on medium-sized muscular vessels, and whether subarachnoid hemorrhage (SAH) is associated with MPA is still unclear. An 85-year-old woman with rapid progressive glomerular nephritis caused by MPA complained of sudden severe headache due to SAH 2 days after admission and subsequently underwent surgery. Cerebrovascular disease occurring simultaneously with MPA might result in poor prognosis, and the complications exacerbate the condition and lead to high mortality; thus, physicians should pay more attention to cerebral aneurysms concurrent with MPA. Among patients with MPA, it is important to identify priority cases and investigate the intracranial vessel environment. To the best of our knowledge, this is a rare report about SAH associated with MPA. We recommend that the presence of cerebrovascular disease should be considered in patients with MPA to improve their prognosis.

Poliangiite microscópica com síndrome pulmão-rim: relato de caso e revisão de literatura / Microscopic polyangiitis with lung-kidney syndrome: case report and literature review

A poliangiite microscópica (PAM) é uma vasculite necrosante sistêmica pauci-imune associada ao anticorpo anticitoplasma de neutrófilos (ANCA) com preferência de pequenos vasos. Relato do caso: Relatamos o caso de uma paciente do sexo feminino, 54 anos, que apresentou quadro de poliartrite migratória em punhos, joelhos e tornozelos associada à rigidez matinal progressiva, com histórico de "rash" malar, fotossensibilidade e alopecia. Progrediu ao longo do ano de 2017 com deterioração da função renal e hemorragia pulmonar, necessitando de cuidados intensivos. A biópsia renal sugeriu padrão compatível com glomerulonefrite pauci-imune e o diagnóstico de poliangiite microscópica foi aventado. Realizou pulsoterapia com metilprednisolona e ciclofosfamida, além de plasmaférese, recebendo alta após estabilização do quadro clínico. Importância do problema: O presente caso ilustra uma complicação incomum e de elevada morbimortalidade da PAM. A negatividade do ANCA dificultou o diagnóstico, sendo necessária a realização de biópsia renal com confirmação diagnóstica. A síndrome pulmão-rim apresenta evolução potencialmente fatal se não instituído precocemente o tratamento. (AU)

Microscopic polyangiitis (MPA) is a pauci-immune systemic necrotizing vasculitis associated with neutrophil anti-cytoplasmic antibody (ANCA) with a preference for small vessels. Case report: We report the case of a 54-year-old woman, who presented migratory polyarthritis in wrists, knees and ankles associated with progressive morning stiffness, with history of malar "rash", photosensitivity and alopecia. It progressed throughout the year of 2017 with deterioration of renal function and pulmonary hemorrhage, requiring intensive care. Renal biopsy suggested a pattern compatible with pauci-immune glomerulonephritis and the diagnosis of microscopic polyangiitis was suggested. She underwent pulse therapy with methylprednisolone and cyclophosphamide, in addition to plasmapheresis, being discharged from hospital after stabilization of the clinical condition. Importance of the issue: The present case reveals an uncommon and high morbimortality complication of MPA. The negativity of the ANCA made diagnosis difficult, and a renal biopsy was necessary to confirm diagnosis. Lung-kidney syndrome is potentially fatal if the treatment is not instituted early. (AU)

Tratamiento exitoso de poliangeítis microscópica con compromiso grave del sistema nervioso central y renal en una paciente anciana / Successful treatment of microscopic polyangiitis with severe compromise of central nervous system and renal in a very elderly patient

RESUMEN El compromiso neurológico del sistema nervioso central (SNC) en las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCAS, del inglés anti-neutrophil cytoplasmic autoantibodies) es raro y potencialmente catastrófico. El estándar de tratamiento ha sido la ciclofosfamida con pulsos de esteroides, sin embargo, este esquema no tiene evidencia fuerte para el compromiso del sistema nervioso central y no está exento de efectos adversos graves sobre todo en la población anciana. En los últimos años, ha aparecido el rituximab como terapia alternativa a la ciclofosfamida para inducir la remisión en este tipo de vasculitis, no obstante, su uso con compromiso neurológico grave también ha sido anecdótico. Se presenta el caso de una paciente de 84 años de edad con poliangeítis microscópica y compromiso neurológico y renal grave, tratada con rituximab evolucionando favorablemente alcanzando la remisión de la enfermedad.

SUMMARY The neurological involvement of the central nervous system (CNS) in vasculitis associated with ANCAS is rare and potentially catastrophic. The standard treatment is cyclophosphamide with pulses of steroids; however, this scheme has no strong evidence for central nervous system involvement and is not free of serious adverse effects especially in the elderly population. In recent year's rituximab has appeared as an alternative therapy to cyclophosphamide to induce remission in this type of vasculitis, however its use with severe neurological involvement has also been anecdotal. We present the case of 84-year-old patient who presented a microscopic polyangiitis with severe neurological and renal involvement, treated with rituximab with a favorable evolution in reaching remission of the disease.

A case of microscopic polyangiitis presenting with acute spinal subdural hemorrhage

This report describes a case of a 62-year-old woman with microscopic polyangiitis (MPA) who developed acute spinal subdural hemorrhage. MPA was confirmed by positive autoantibodies to myeloperoxidase and focal segmental necrotizing and pauci-immune crescentic glomerulonephritis on renal biopsy. She did not recover from paraplegia due to acute spinal subdural hemorrhage, despite decompression operation and aggressive immunosuppression. Although spontaneous spinal hemorrhage in MPA patients is very rare, the prognosis for such patients is poor. Considering the possibility of ongoing vasculitis activity in extra-renal organs, clinicians should be very cautious to attenuate the strength of immunosuppressant drugs, even in patients with chronic or irreversible renal pathology.

Delta Neutrophil Index Is Associated with Vasculitis Activity and Risk of Relapse in ANCA-Associated Vasculitis

PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA). We collected clinical and laboratory data including BVAS, five factor score (FFS), and DNI. The correlation coefficient and cumulative relapse free survival rate were obtained. The optimal cut-off of DNI was extrapolated by calculating the area under the receiver operator characteristic curve. RESULTS: DNI was significantly related to cross-sectional BVAS. Furthermore, among continuous variables, only DNI could reflect BVAS of GPA and MPA, but not EGPA. Severe AAV was defined as BVAS ≥20 (the highest quartile). At diagnosis, patients having DNI ≥0.65% had a significantly higher risk of severe GPA and MPA than those having not (relative risk 4.255) at diagnosis. During the follow-up, DNI ≥0.65% could predict the higher relapse rate. CONCLUSION: DNI could reflect BVAS at diagnosis and furthermore, DNI ≥0.65% could not only identify severe AAV at diagnosis, but also predict relapse during the follow-up in patients with GPA and MPA.