The value of repeat kidney biopsy in lupus nephritis. A systematic review / La utilidad de la biopsia renal repetida en nefritis lúpica. Una revisión sistemática

ABSTRACT A renal biopsy is the 'gold standard' for diagnosis and classification of lupus nephritis (LN). The role of repeat renal biopsy in lupus nephritis (LN) to guide treatment or predict prognosis has been controversial. A systematic literature review was conducted based on retrospective and prospective studies. The studies were identified using English electronic scientific databases, including MEDLINE PUBMED, published between January 1990 and August 2020. The eligibility criteria were studies including adult LN patients with at least one follow-up renal biopsy with appropriate longitudinal information. Case reports, studies with incomplete information or including duplicate patients were excluded. Based on the inclusion and exclusion criteria, a total of 73 publications were identified. This study included a total of 1167 repeat biopsies in LN patients from 15 studies. The primary indication for a repeat biopsy was relapse in 44-78% of the cases, and lack of response in 13-51%. Additionally, several repeat biopsies were done according to the protocol, after induction and maintenance therapy. In terms of histopathological class switches, there was a higher frequency of changes from nonproliferative to proliferative lesions. Only two studies provide a definition of histological response. There were often changes in the therapeutic approach after a repeat biopsy. Repeat kidney biopsies are helpful in patients with LN flare/relapse, and in patients with poor treatment response. Histological transformation was a common finding. The histologic and clinical responses are discordant. A repeat biopsy could be of prognostic value for therapeutic decision-making.

RESUMEN La biopsia renal es el «estándar de oro¼ para el diagnóstico y la clasificación de la nefritis lúpica (NL). El papel de la biopsia renal repetida en nefritis lúpica para orientar el tratamiento o predecir el pronóstico ha sido controversial. Se llevó a cabo una revisión sistemática de la literatura basada en estudios retrospectivos y prospectivos. Los estudios se identificaron a través de bases de datos científicas electrónicas en inglés, incluyendo Medline PubMed, de publicaciones entre enero de 1990 y agosto del 2020. Los criterios de elegibilidad fueron estudios que incluyeran a pacientes adultos con NL, quienes tuvieran al menos una biopsia renal de seguimiento, con información longitudinal apropiada. Se excluyeron informes de casos, estudios con información incompleta o con pacientes duplicados. Basándose en los criterios de inclusión y exclusión, se identificaron 73 publicaciones. En la presente revisión se analizaron un total de 1.167 biopsias repetidas en pacientes con NL en 15 estudios. Las principales indicaciones para la biopsia repetida fueron: recidiva en 44-78% de los casos, y falta de respuesta en 13-51%. Adicionalmente, varias biopsias repetidas se hicieron conforme al protocolo, luego de la terapia de inducción y de mantenimiento. Con respecto a los cambios de clase histopatológica, hubo una mayor frecuencia de cambios de lesiones no proliferativas a lesiones proliferativas. Solamente dos estudios ofrecen una definición de respuesta histológica. Con frecuencia hubo cambios en el abordaje terapéutico después de realizar la biopsia repetida. Las biopsias renales repetidas son útiles en pacientes con exacerbación/recidiva y en pacientes con falta de respuesta a tratamiento. La transformación histológica fue un hallazgo frecuente; las respuestas histológicas y clínicas son discordantes. Una biopsia repetida puede ser de valor pronóstico para la toma de decisiones terapéuticas.

Anti-phosphatidylserine/prothrombin antibodies in patients with polyarteritis nodosa.

INTRODUCTION: Anti-phospatidylserine/prothrombin (aPS/PT) antibodies have been described in cutaneous Polyarteritis Nodosa (PAN) in association with specific manifestations. OBJECTIVES: To determine aPS/PT antibodies in patients with PAN and its correlation with clinical manifestations. METHODS: Cross-sectional comparative study including PAN patients and 20 controls (10 Microscopic Polyangiitis [MPA] and 10 Behçet's disease [BD]). Clinical and demographic variables, treatment, serologic markers, prognosis, activity and damage indexes were evaluated. aPS/PT, anti-cardiolipin (aCL), anti-beta 2 glycoprotein 1 (anti-B2GP1) antibodies, and lupus anticoagulant (LA) were determined. RESULTS: Fourteen patients with PAN were included, 11 (79%) women, with disease duration of 207 months, and mostly inactive disease. Only one patient with PAN and one with BD were positive for aPS/PT IgG. LA was the most frequent antibody identified. One patient with MPA and one with BD were positive for aCL IgM; one with MPA for anti-B2GP1 IgG, and one with PAN for anti-B2GP1 IgM. CONCLUSIONS: aPS/PT antibodies are not frequent in patients with longstanding inactive PAN.

Anticuerpos antifosfatidilserina/protrombina en pacientes con poliarteritis nodosa / Anti-Phosphatidylserine/Prothrombin antibodies in patients with Polyarteritis Nodosa

Antecedentes: Anticuerpos antifosfatidilserina/protrombina (aPS/PT) han sido descritos en poliarteritis nodosa (PAN) cutánea, en asociación con manifestaciones específicas. Objetivos: Determinar anticuerpos aPS/PT en pacientes con PAN y analizar su correlación con manifestaciones clínicas. Métodos: Estudio transversal comparativo de pacientes con PAN y 20 controles (10 con poliangitis microscópica [PAM] y 10 con enfermedad de Behçet [EB]). Se evaluaron variables demográficas, clínicas, serológicas y tratamiento; índices de pronóstico, actividad y daño. Se determinaron anticuerpos aPS/PT, anticardiolipina (aCL), anti-beta 2 glicoproteína 1 (anti-B2GP1) y anticoagulante lúpico (AL). Resultados: Fueron incluidos 14 pacientes con PAN, 11 (79%) mujeres, con duración de la enfermedad de 207 meses, y principalmente enfermedad inactiva. Sólo un paciente con PAN y uno con EB fueron positivos para aPS/PT IgG. El anticuerpo antifosfolípido más frecuente fue AL. Un paciente con PAM y uno con EB fueron positivos para aCL IgM; uno con PAM para anti-B2GP1 IgG, uno con PAN para anti-B2GP1 IgM. Conclusiones: Los anticuerpos aPS/PT son infrecuentes en pacientes con PAN inactiva de larga evolución.(AU)

Introduction: Anti-phosphatidylserine/prothrombin (aPS/PT) antibodies have been described in cutaneous Polyarteritis Nodosa (PAN) in association with specific manifestations. Objectives: To determine aPS/PT antibodies in patients with PAN and its correlation with clinical manifestations.Methods: Cross-sectional comparative study including PAN patients and 20 controls (10 Microscopic Polyangiitis [MPA] and 10 Behçet's disease [BD]). Clinical and demographic variables, treatment, serological markers, prognosis, activity and damage indexes were evaluated. aPS/PT, anti-cardiolipin (aCL), anti-beta 2 glycoprotein 1 (anti-B2GP1) antibodies, and lupus anticoagulant (LA) were determined. Results: Fourteen patients with PAN were included, 11 (79%) women, with disease duration of 207 months, and mostly inactive disease. Only one patient with PAN and one with BD were positive for aPS/PT IgG. LA was the most frequent antibody identified. One patient with MPA and one with BD were positive for aCL IgM; one with MPA for anti-B2GP1 IgG, and one with PAN for anti-B2GP1 IgM. Conclusions: aPS/PT antibodies are not frequent in patients with longstanding inactive PAN.(AU)

Clinical Characteristics and Outcomes of Mexican Patients With Polyarteritis Nodosa: A Single-Center Study Over 40 Years.

METHODS: We conducted a medical records review study (1975-2018) that included patients with childhood- and adult-onset PAN. Demographics, organ involvement, phenotype, treatment, and outcomes were assessed and compared against previously published cohorts. RESULTS: Thirty-one patients were included, 20 (64.5%) female, with a median age at diagnosis of 28 years (interquartile range [IQR], 16-42). Seven (23%) were classified as cutaneous; 23 (74%), systemic; and 1 (3%), progressive systemic phenotype. Eleven patients (35%) had childhood-onset PAN. Most common manifestations were musculoskeletal (71%), cutaneous (68%), constitutional (61%), peripheral neuropathy (39%), and gastrointestinal (29%). The median Birmingham Vasculitis Activity Score and Five-Factor Score at diagnosis were 9 (IQR, 4-13) and 1 (IQR, 0-1), respectively. Most patients were treated with glucocorticoids (94%). Twenty-four (80%) achieved complete and 6 (20%) partial remission at a median follow-up time of 30 months (8-192 months). The median Vasculitis Damage Index at last follow-up was 1 (IQR, 0-1). Nineteen (66%) experienced relapses. Patients with childhood-onset PAN more frequently had central nervous system and gastrointestinal involvement (36% vs 5%, p = 0.04 and 64% vs 10%, p = 0.003, respectively), microaneurysms (100% vs 38%, p = 0.02), and lower levels of C-reactive protein (0.3 vs 15.4 mg/dL, p = 0.03), compared with adult-onset PAN patients. CONCLUSIONS: Our cohort of PAN patients showed predominantly a systemic phenotype. Outcomes were generally good, with most patients achieving complete remission. Childhood-onset differed from adult-onset PAN in terms of clinical and serological characteristics, whereas clinical manifestations and outcomes may be different than the ones reported in other cohorts.

Anti-Phosphatidylserine/Prothrombin antibodies in patients with Polyarteritis Nodosa. / Anticuerpos antifosfatidilserina/protrombina en pacientes con poliarteritis nodosa.

INTRODUCTION: Anti-phosphatidylserine/prothrombin (aPS/PT) antibodies have been described in cutaneous Polyarteritis Nodosa (PAN) in association with specific manifestations. OBJECTIVES: To determine aPS/PT antibodies in patients with PAN and its correlation with clinical manifestations. METHODS: Cross-sectional comparative study including PAN patients and 20 controls (10 Microscopic Polyangiitis [MPA] and 10 Behçet's disease [BD]). Clinical and demographic variables, treatment, serological markers, prognosis, activity and damage indexes were evaluated. aPS/PT, anti-cardiolipin (aCL), anti-beta 2 glycoprotein 1 (anti-B2GP1) antibodies, and lupus anticoagulant (LA) were determined. RESULTS: Fourteen patients with PAN were included, 11 (79%) women, with disease duration of 207 months, and mostly inactive disease. Only one patient with PAN and one with BD were positive for aPS/PT IgG. LA was the most frequent antibody identified. One patient with MPA and one with BD were positive for aCL IgM; one with MPA for anti-B2GP1 IgG, and one with PAN for anti-B2GP1 IgM. CONCLUSIONS: aPS/PT antibodies are not frequent in patients with longstanding inactive PAN.

Características clínicas de pacientes con osteoporosis del consultorio de especialidades, Instituto Salvadoreño del Seguro Social / Clinical characteristics of patients with osteoporosis from the specialty office, "Instituto Salvadoreño del Seguro Social"

La osteoporosis es la enfermedad metabólica ósea más frecuente, responsable de la mayor parte de las fracturas que se producen en personas mayores de 50 años. Un manejo integral para la osteoporosis incluye la evaluación de las personas en mayor riesgo, exclusión de causas secundarias de baja densidad mineral ósea, y la selección del tratamiento adecuado. Actualmente, se estima que más de 200 millones de personas en todo el mundo tienen osteoporosis. En nuestro país, así como en la mayoría de los países latinoamericanos, la osteoporosis no está considerada ni reconocida como un problema de salud pública, pese a la carga que significan las fracturas por fragilidad en los sistemas de salud. No se cuenta con estudios de las características clínicas y prevalencia de la población con osteoporosis en el Instituto Salvadoreño del Seguro Social. Se identifica las características clínicas, factores de riesgo y valores de densitometría para clasificar posteriormente a la población en grupos de riesgo, ya que en base a ello se orientan las intervenciones terapéuticas. El número de pacientes con diagnóstico confirmado de osteoporosis en control con Endocrinología en el año 2014 fue de 240, del cual se estudió a 148 pacientes de forma aleatoria, donde el sexo femenino corresponde el 97.3% de la población estudiada, la edad media fue de 65.8 +/- 9.6 años; el principal tipo de osteoporosis fue la III o secundaria. Las enfermedades asociadas más frecuentes son hipertensión arterial (45.9%), hipotiroidismo (39.8%) y diabetes mellitus (20.2%). Los factores de riesgo más frecuentes son la edad mayor de 65 años (56.7%), antecedentes de fracturas previas y antecedentes familiares de fractura de cadera. Las causas más frecuentes de osteoporosis secundaria son diabetes mellitus (20.2%), deficiencia de vitamina D (16.2%), amenorrea de larga evolución (8.1%) y artritis reumatoide (8.1%). Según la ponderación FRAX el 87% de la población tienen bajo riesgo de fractura osteoporótica mayor a 10 años; y el 51.7% de alto riesgo para fractura de cadera a 10 años. Conclusión: la osteoporosis es más frecuente en el sexo femenino, la mitad de los pacientes son mayores de 65 años. El tipo de osteoporosis más frecuente es la secundaria y sus principales causas fueron diabetes mellitus, deficiencia de vitamina D, amenorrea de larga evolución y artritis reumatoide. Más de la mitad de la población presenta riesgo alto para fractura de cadera a 10 años