Testicular infarction due to polyarteritis nodosa: a case report and literature review / 现代泌尿外科杂志

【Objective】 To report a case of testicular infarction due to polyarteritis nodosa (PAN), and to discuss its clinical diagnosis and treatment based on relevant literatures at home and abroad, so as to have a better understanding of this rare disease. 【Methods】 Clinical data of a case complaining of scrotal pain who was initially diagnosed as testicular torsion and later confirmed to be testicular infarction due to PAN were retrospectively analyzed, and relevant literatures were reviewed. 【Results】 With glucocorticoid, vasodilator and antioxidant treatment, the patient’s testicular blood flow was improved. 【Conclusion】 Testicular infarction due to PAN is a rare disease which is difficult to diagnose timely. The diagnosis depends on biopsy and the standards formulated by American College of Rheumatology (ACR). Good prognosis can be achieved with timely diagnosis and correct treatment.

Cutaneous polyarteritis nodosa: An atypical simulator. Report of 3 cases and a narrative review of the literature

Objectives: To report the clinical manifestations and therapeutic schemes established in three patients with cutaneous polyarteritis nodosa, as well as to describe the pathophysiology, clinical features, diagnostic criteria, and therapeutic options. Methods: A literature review was performed using Google scholar and PubMed and MeSH terms. There was no limit on the publication date or language for the selection of the articles. Results: Cutaneous polyarteritis nodosa is an uncommon small and medium-sized vessel vasculitis, and is rarely reported in Colombia. Although its pathophysiology is unknown, it is considered to be mediated by the deposition of immunocomplexes in the walls of blood vessels. It can be triggered by infectious agents and be associated with autoimmune diseases. The cutaneous manifestations mainly include subcutaneous painful nodules, livedo reticularis, and ulcers. Myalgia, arthralgia, peripheral neuropathy, and fever can also be present. It represents a diagnostic challenge. Treatment is not standardized and is guided according to the severity. Conclusions: Cutaneous polyarteritis nodosa is a rare entity, with a wide variety of cutaneous manifestations. There is still no specific diagnostic test. Its diagnosis represents a challenge for the dermatologist, and requires multidisciplinary management, in which the rheumatologist plays a fundamental role.

Objetivos: Reportar las diferentes manifestaciones clínicas y los esquemas terapéuticos instaurados en 3 pacientes con poliarteritis nudosa cutánea. Así mismo, describir la fisiopatología, el cuadro clínico, los criterios diagnósticos y las opciones terapéuticas. Métodos: Se realizó una búsqueda de la literatura en Google Académico y PubMed utilizando términos MeSH. En la selección de la bibliografía, la búsqueda no se limitó por fecha de publicación ni por idioma, debido al escaso número de reportes. Resultados: La poliarteritis nudosa cutánea es una vasculitis de pequeño y mediano vaso, infrecuente, poco reportada en Colombia. Su fisiopatología no es del todo conocida, se considera mediada por el depósito de inmunocomplejos en las paredes de vasos sanguíneos; puede ser desencadenada por agentes infecciosos y asociarse con enfermedades autoinmunes. Las manifestaciones cutáneas incluyen principalmente nódulos subcutáneos dolorosos, livedo reticular y úlceras. A nivel extracutáneo podrían encontrarse mialgias, artralgias, neuropatía periférica y fiebre. El diagnóstico representa un desafío. El tratamiento no está estandarizado y es guiado según la severidad de la patología. Conclusión: La poliarteritis nudosa cutánea es una entidad poco frecuente, con una amplia variedad de manifestaciones cutáneas. Hasta el momento, no contamos con una prueba diagnóstica específica. Su diagnóstico representa un reto para el dermatólogo y requiere un manejo multidisciplinario, en el cual el reumatólogo desempeña un papel fundamental.

Poliarteritis nodosa sistémica / Systemic polyarteritis nodosa

Resumen La poliarteritis nodosa sistémica de inicio en la infancia es una vasculitis caracterizada por inflamación y necrosis fibrinoide de las arterias de mediano y pequeño calibre, con cuadro clínico extraordinariamente variable y dependiente de la localización histológica de la lesión. Su compromiso cardiaco constituye un comportamiento infrecuente en pediatría, por lo que se considera de interés presentar el caso de un escolar de 8 años, determinado por compromiso hemodinámico secundario a derrame pericárdico, disfunción sistólica grave y múltiples dilataciones aneurismáticas, que permite establecer la sospecha de vasculitis de vasos pequeños y medianos. Se realiza un diagnóstico oportuno gracias al cual se evitan complicaciones potencialmente mortales y se instaura un tratamiento con buena respuesta a corto, mediano y largo plazo.

Abstract Systemic polyarteritis nodosa of childhood onset is a vasculitis characterized by inflammation and fibrinoid necrosis in the medium and small-caliber arteries, with an extraordinarily variable clinical picture and dependent on the histological location of the lesion. His cardiac involvement constitutes an infrequent behavior in pediatrics, so it is considered of interest to present the case of an 8-year-old schoolboy, characterized by hemodynamic compromise secondary to pericardial effusion, severe systolic dysfunction, and multiple aneurysmal dilatations that establish the suspicion of vasculitis. small and medium glasses. A timely diagnosis is made avoiding life-threatening complications and allowing to establish a treatment with a good response in the short, medium and long term.

Perfil demográfico y clínico de la poliarteritis nodosa sistémica y cutánea en un grupo de pacientes pediátricos de Medellín, 2010-2019 / Demographic and clinical profile of systemic and cutaneous Polyarteritis nodosa in a group of pediatric patients in Medellin, 2010-2019

Resumen Objetivo: describir las principales características demográficas y clínicas de los pacientes pediátricos con poliarteritis nodosa (PAN). Metodología: estudio descriptivo retrospectivo. Resultados: se obtuvieron las historias clínicas de menores de 18 años con diagnóstico de vasculitis o PAN atendidos entre el 2010 y 2019 en dos hospitales de Medellín. Se incluyeron 19 pacientes, 11 clasificados como PAN cutánea (PANC) según la definición de la Liga Europea contra el Reumatismo y la Sociedad Europea de Reumatología Pediátrica, y 8 como PAN sistémica (PANS) acorde con los criterios de Ankara 2008. El 57,9 % fueron varones (n = 11). La mediana de la edad en el diagnóstico fue 10 años (3-17 años) y del tiempo de seguimiento: 15 meses (0-105 meses). Las manifestaciones más frecuentes fueron nódulos subcutáneos (84,2 %), fiebre (57,9 %), artralgias (57,9 %) y dolor en las pantorrillas (52,6 %). Los pacientes con PANS presentaron más pérdida de peso y hemoglobina baja. 5 niños cursaron con úlceras (26,3 %) cuatro con PANC; 2 exhibieron necrosis lingual (10,5 %) y 2 necrosis digital (10,5 %), todos con PANC. El promedio de la PCR fue de 10,2 mg/dl (0,33-45,3) y, de la eritrosedimentación: 74 mm/hora (15-127 mm/hora). En las imágenes hubo un realce meníngeo, aneurismas intrahepáticas y mesentéricos, entre otros hallazgos. Todos recibieron esteroides sistémicos. Ninguno falleció. Conclusión: en esta serie, la PAN pediátrica fue más común en niños de edad escolar. La PANC fue más frecuente que la PANS. Las manifestaciones más descritas fueron los nódulos subcutáneos, las mialgias, la fiebre, las artralgias y el dolor en las pantorrillas. Aunque se ha considerado la PANC como una enfermedad benigna, estos pacientes pueden requerir tratamiento con glucocorticoides e inmunosupresores.

Summary Objective: To describe the main demographic and clinical characteristics of patients who were diagnosed with childhood polyarteritis nodosa (PAN). Methods: A descriptive study was conducted using retrospective data Results: The clinical registries from patients under 18th years with vasculitis or PAN diagnosis attended between 2010 and 2019 in two Medical Centers from Medellín were obtained. Nineteen patients were included: eleven classified as cutaneous PAN (PANC) according to the definition of the European League Against Rheumatism and the European Society of Pediatric Rheumatology and eight as systemic PAN according to Ankara 2008 Criteria. 57.9% were male. The median age at diagnosis was ten years (3-17), and the median follow-up period was 15 months (0-105). The most common clinical characteristics were subcutaneous nodules (84.2%), fever (57.9%), arthralgias (57.9%), and calf pain (52.6%). SPAN patients had more frequent weight loss and lower hemoglobin. Five patients had ulcers (26.3%), four of them with CPAN; two patients had lingual necrosis (10.5%), and two digital necrosis (10.5%), all of them also with CPAN. CRP mean was 10.2 mg/dl (0.33-45.3) and mean erythrocyte sedimentation rate (ESR) was 74 mm/h (15-127 mm/h). Meningeal enhancing, intrahepatic, and mesenteric aneurism, among others, were reported in diagnostic images. All patients required treatment with systemic glucocorticoid. None of the patients died during the follow-up period. Conclusions: In this pediatric series from Medellín-Colombia, PAN was more common in boys of school age. CPAN was more frequent than SPAN. The clinical characteristics most reported were subcutaneous nodules, myalgias, arthralgias, and calf pain. Although CPAN has been considered a benign disease, these patients may require systemic glucocorticoid and immunosuppressive therapy.

Recommendations of diagnosis and treatment of polyarteritis nodosa / 中华内科杂志

Polyarteritis nodosa (PAN) is a rare vasculitis that mainly involves small and medium arteries. It often occurs at the points where the vessels bifurcate, leading to microaneurysm formation, thrombosis, aneurysm rupture and bleeding, and infarction of organs.About a third of cases are associated with hepatitis B virus (HBV) infection.All tissues and organs of the body can be affected, with skin, joints and peripheral nerves being the most common.The pathological changes were fibrinoid necrosis, inflammatory cell infiltration and luminal thrombosis in the acute stage, and fibrous hyperplasia in the chronic stage.Overall outcomes for the disease have improved in recent decades, mainly reflecting early diagnosis and more effective treatments.The main treatments for PAN are glucocorticoid and cyclophosphamide.Patients with HBV-associated PAN should receive antiviral therapy and plasma exchange.

A case report of polyarteritis nodosa accompany with renal multiple aneurysms rupture hemorrhage / 中华泌尿外科杂志

A case of polyarteritis nodosa was reviewed. The patient was a 58-year-old middle-aged man. He developed fever, headache, fatigue and other symptoms. Three weeks after, massive hematuria was seen. CT plain scan and MRI indicated blood clots in renal pelvis, and pericardium and pleural effusion. Renal arteriography indicated multiple venous fistula in both kidneys. Enhanced CT scan showed multiple small aneurysms in abdominal aortic branches, bilateral renal arteries and pancreatic arteries. The diagnosis of nodular polyarteritis was confirmed. Glucocorticoid and immunosuppressive agents were given. Meanwhile, highly selective renal arteriovenous fistula embolization was performed, Postoperative hematuria was effectively controlled, and the general condition was gradually improved. The patient was followed up for 2 months, and the condition was stable.

Ergotism, a relatively unknown mimic of vasculitis: A case report and review of literature / Ergotismo: un imitador poco conocido de vasculitis. Reporte de caso y revisión de la literatura

ABSTRACT Introduction: Ergotism is a vasospasm that affects visceral and peripheral muscle arteries. Classically, symmetrical involvement of lower limb arteries is described, and is often associated with a history of chronic consumption of ergotamine derived medications (Cafergot). Case report: A 22 year-old healthy man with infectious mononucleosis syndrome, who presented with a sudden onset of paraesthesias in the lower limbs, as well as livedo reticularis. The initial diagnosis was a medium-sized vessel vasculitis (polyarteritis nodosa). The symptoms were preceded by the administration of Cafergot for headache treatment, and resolved spontaneously. The magnetic resonance angiography (MRA) of the lower limbs showed occlusion of peroneal arteries, with filiform distal flow. Other infectious, autoimmune and cardiovascular origins were ruled out. Discussion: Ergotism is an important differential diagnosis in the study of the patient with vasculitis, especially in acute onset presentations. Its treatment is the suspension of the causal drug, with vasodilator and surgical vascular procedures, if necessary. Conclusions: Ergotism is an imitator of vasculitis, especially in young patients with a history of difficult to control migraine. The concomitant administration of CYP3A4 inhibitors (mainly, protease inhibitors and macrolides) enhances the toxic effects of ergot.

RESUMEN Introducción: El ergotismo es un vasoespasmo que afecta las arterias musculares periféricas y viscerales. Clásicamente se describe la afectación simétrica de las arterias de las extremidades inferiores, a menudo asociada con el consumo crónico de medicamentos derivados de ergotamina (Cafergot®). Caso clínico: Varón sano de 22 anos con síndrome de mononucleosis, presentó parestesias en las extremidades inferiores y livedo reticularis de forma súbita, el diagnóstico inicial fue una vasculitis de mediano vaso (poliarteritis nodosa). Los síntomas fueron precedidos por la administración de Cafergot® para el tratamiento de cefalea, y se resolvieron espontáneamente. La angiografía por resonancia magnética (ARM) de las extremidades inferiores mostró oclusión de las arterias peroneas, con flujo distal filiforme. Se descartaron otras etiologías infecciosas, autoinmunes y cardiovasculares. Discusión: Los ergotismos son un diagnóstico diferencial importante en el estudio del paciente con vasculitis, especialmente en presentaciones de inicio agudo. Su tratamiento es la suspensión del fármaco causal, vasodilatadores y procedimientos vasculares quirúrgicos, si es necesario. Conclusiones: El ergotismo es un imitador de vasculitis, especialmente en pacientes jóvenes con antecedentes de migrana de difícil control. La administración concomitante de inhibidores del CYP3A4 (principalmente, inhibidores de proteasa y macrólidos) potencia los efectos tóxicos del ergot.

Poliarterite nodosa e osteonecrose em paciente feminino como afecção rara em atenção primária à saúde / Nodosa polyarteritis and osteonecrosis in a female patient as a rare condition in primary health care

A poliartrite nodosa (PAN) faz parte de um grupo de patologias autoimunes caracterizadas pela vasculite. Quando associada à osteonecrose, tal comorbidade torna-se ainda mais rara, sendo mais comum em indivíduos do sexo masculino e com manifestações clínicas variadas. O presente estudo trata-se de um relato de caso de uma paciente de 40 anos do sexo feminino que apresentou PAN, a qual se agravou desenvolvendo osteonecrose na articulação coxofemoral bilateral. O tratamento por meio da terapia com corticoides associados a ciclofosfamida, apesar de necessitar monitorização frequente, tem se mostrado eficaz na redução do impacto da morbidade sobre o bem estar do paciente. O quadro apresenta um prognóstico complexo e de difícil reconhecimento visto sua raridade, sintomatologia variada e diagnóstico histopatológico.

Polyarteritis nodosa (PAN) is part of a group of autoimmune disorders characterized by vasculitis. When associated with osteonecrosis, such comorbidity becomes even rarer, being more common in males. Its clinical manifestations are varied. The present study is a case report of a 40-year-old female patient who presented with PAN, which was aggravated by developing osteonecrosis on the bilateral hip joint. Treatment with corticosteroid therapy associated with cyclophosphamide, despite requiring frequent monitoring, has been shown to be effective in reducing the impact of morbidity on patient's well-being. The condition has a complex prognosis and is difficult to recognize due to its rarity, varied symptoms, and histopathological diagnosis.

Compromiso vesical en paciente pediátrico con poliarteritis nodosa: primera descripción / Bladder involvement in a paediatric patient with polyarteritis nodosa: First description

RESUMEN La poliarteritis nudosa (PAN) es una vasculitis necrosante, rara en la infancia, caracterizada por el compromiso de vasos pequeños/medianos y de múltiples órganos. Presentamos a una paciente que inició a los 4 arios con síndrome febril prolongado, dolor abdominal crónico, mialgias incapacitantes y compromiso en la piel, quien luego de 2 años de cuadro clínico completa criterios clínicos para PAN. Recibió tratamiento con corticoide sistêmico por vía oral e intravenosa, 6 meses de ciclofosfamida por vía intravenosa y manejo de mantenimiento con inmunosupresores convencionales sin respuesta adecuada, logrando control de la enfermedad únicamente con ciclofosfamida por vía oral y corticoide a largo plazo. Luego de 5 años y de recibir una dosis alta acumulada de ciclofosfamida, inicia con cuadros de hematuria macroscópica. Se evaluaron, entre otras causas, la toxicidad por ciclofosfamida y la actividad de la enfermedad. El estudio incluyó biopsia vesical, con hallazgo de vas-culitis necrosante de paredes vesicales. La vasculitis vesical es raramente reportada en la literatura (3-5 casos en adultos) y en lo consultado no hay reportes en niños. Se describe, en nuestro conocimiento, el primer caso de compromiso vesical asociado a vasculitis sistêmica reportado en la edad pediátrica.

A B S T R A C T Polyarteritis nodosa (PAN) is a necrotising vasculitis, rare in childhood, and characterized by the inflammation of small and medium vessels and multiple organ involvement. The case is presented of a 4 year old girl with prolonged febrile syndrome, chronic abdominal pain, disabling myalgia, and skin involvement. After 2years of symptoms, she met clinical criteria for PAN. She received treatment with oral and intravenous systemic corticosteroids, 6 months of intravenous cyclophosphamide and maintenance with conventional immuno-suppressants without an adequate response. However, she showed clinical improvement with oral cyclophosphamide and long-term corticosteroids. She had several relapses during follow-up visits due to irregular treatment requiring a high cumulative dose of cyclophosphamide. Five years later she presented with macroscopic haematuria, and was assessed for, among other causes, cyclophosphamide toxicity and disease activity. The workup included cystoscopy and bladder biopsy with findingof necrotising vasculitis of bladder wall. Bladder vasculitis is rarely reported in the literature (3-5 cases in adults) and in that consulted there are no reports in children. To our knowledge, this is the first case of bladder involvement associated with systemic vasculitis reported in the paediatric age.

Polyarteritis nodosa / Periarteritis nodosa

Abstract Polyarteritis nodosa was first described in 1866 by Zarco. Histologically, it is characterized by necrosis of the medium-sized arteries. Clinical case: A 63-year-old patient presented with myalgia, blurred vision, paresthesias and loss of muscle strength. On physical exam, he had hypesthesia in the left foot. Paraclinical studies ruled out small vessel vasculitis, and, in the end, the biopsy was compatible with polyarteritis nodosa. Discussion: this is a low prevalence entity with widely variable clinical manifestations. Therefore, the American College of Rheumatology criteria must be used for diagnosis, keeping in mind that the gold standard is histopathology. It is treated with immunosuppressants and the patient's prognosis is determined through the assessment of four criteria.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1388).

Resumen La poliarteritis nodosa fue descrita por primera vez en 1866 por Zarco. Histológicamente se caracteriza por la presencia de necrosis de las arterias de mediano calibre. Caso clínico: paciente de 63 años con mialgias, visión borrosa, parestesias y pérdida de fuerza muscular. Al examen físico con hipoestesia en pie izquierdo. Los paraclínicos descartaron vasculitis de pequeño vasos, finalmente la biopsia era compatible con poliarteritis nodosa. Discusión: es una entidad de baja prevalencia, la cual tiene una amplia variabilidad en cuanto a manifestaciones clínicas, por lo cual es necesario usar los criterios del Colegio Americano de Reumatología para el diagnóstico, teniendo en cuenta que el gold estándar es el estudio histopatológico. El tratamiento se da con medicamentos inmunosupresores y el pronóstico del paciente se establece mediante la evaluación de cuatro criterios.(Acta Med Colomb 2020; 45. DOI:http://doi.org/10.36104/amc.2020.1388).

Is macular lymphocytic arteritis limited to the skin? Long-term follow-up of seven patients

Abstract Background: Macular lymphocytic arteritis most commonly presents as hyperpigmented macules on the lower limbs. The pathogenesis of this disease is still unclear and there is an ongoing debate regarding whether it represents a new form of cutaneous vasculitis or an indolent form of cutaneous polyarteritis nodosa. Objective: To describe clinical, histopathological, and laboratory findings of patients with the diagnosis of macular lymphocytic arteritis. Methods: A retrospective search was conducted by reviewing cases followed at the Vasculitis Clinic of the Dermatology Department, School of Medicine, University of São Paulo, between 2005 and 2017. Seven patients were included. Results: All cases were female, aged 9-46 years, and had hyperpigmented macules mainly on the legs. Three patients reported symptoms. Skin biopsies evidencing a predominantly lymphocytic infiltrate affecting arterioles at the dermal subcutaneous junction were found, as well as a typical luminal fibrin ring. None of the patients developed necrotic ulcers, neurological damage, or systemic manifestations. The follow-up ranged from 18 to 151 months, with a mean duration of 79 months. Study limitations: This study is subject to a number of limitations: small sample of patients, besides having a retrospective and uncontrolled study design. Conclusions: To the best of the authors' knowledge, this series presents the longest duration of follow-up reported to date. During this period, none of the patients showed resolution of the lesions despite treatment, nor did any progress to systemic vasculitis. Similarities between clinical and skin biopsy findings support the hypothesis that macular lymphocytic arteritis is a benign, incomplete, and less aggressive form of cutaneous polyarteritis nodosa.

Report of a case of probable overlap between systemic erythematous lupus and polyarteritis nodosa / Reporte de un caso de probable overlap de lupus eritematoso sistémico con panarteritis nudosa

ABSTRACT Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease with a wide range of clinical manifestations that may affect any organ. Polyarteritis nodosa (PAN) is defined as necrotizing inflammatory changes in the medium and small vessels, a rare form of systemic necrotizing vasculitis in childhood. This article discusses the case of a patient with a history of deep venous thrombosis of the left leg, who presented with erythematosus purple lesions in her right hand, associated with pain, intermittent claudication, progressive limping and generalized edema. While in hospital, she was diagnosed with SLE with renal involvement and medium vessel vasculitis mainly of the upper limbs. She also met the criteria for PAN, a rare association that is seldom described in the medical literature.

RESUMEN El lupus eritematoso sistémico (LES) es una enfermedad autoinmune heterogénea con una amplia variedad de manifestaciones clínicas que pueden afectar cualquier órgano. La panarteritis nudosa (PAN) se define como cambios inflamatorios necrotizantes en arterias medianas o pequenas, siendo una vasculitis necrotizante sistêmica rara en la infancia. Presentamos el caso de una paciente con antecedente de trombosis venosa profunda del miembro inferior izquierdo, que presenta lesiones violáceas eritematosas en la mano derecha, asociadas a dolor, claudicación intermitente, limitación funcional progresiva, así como edema generalizado. Durante la hospitalización se llega al diagnóstico de LES con compromiso renal y vasculitis de vasos medianos con predominio de miembros superiores que cumple criterios de PAN, asociación rara muy poco descrita en la bibliografía.

Multifocal myopathy in a patient with polyarteritis nodosa; usefulness of magnetic nuclear resonance as a diagnostic test / Miopatia multifocal en paciente con poliarteritis nodosa: utilidad de la resonancia nuclear magnética como prueba diagnóstica

ABSTRACT Polyarteritis nodosa is part of the primary systemic vasculitis that specifically compromises vessels of medium caliber, and can affect virtually any organ. The diagnosis of this disease is based on clinical criteria, such as pain and weakness in the lower limbs, as well as laboratory results and the histology report that shows necrotizing, segmental and focal inflammation of the vessels involved. The case is presented of a 42 year-old woman with a previous diagnosis of polyarteritis nodosa, who, 12 years later, presented with an episode of activation of the disease associated with venous thrombosis and multifocal myopathy in the lower limbs. This is a rare presentation that should be suspected in this type of patients.

RESUMEN La poliarteritis nodosa hace parte de las vasculitis sistémicas primarias, específicamente compromete vasos de mediano calibre pudiendo afectar virtualmente a cualquier órgano. El diagnóstico de esta enfermedad se basa en criterios clínicos, como el dolor y la debilidad en los miembros inferiores, además de reportes paraclínicos y el compromiso histológico, que evidencia inflamación necrosante, segmentaria y focal de los vasos involucrados. A continuación, presentamos el caso clínico de una mujer de 42 arios, con diagnóstico previo de poliarteritis nodosa, que presenta, 12 años después, un episodio de activación de la enfermedad asociado a trombosis venosa y miopatía multifocal en miembros inferiores, una presentación poco frecuente pero que debe sospecharse en este tipo de pacientes.

Clinical characteristics of 57 patients with polyarteritis nodosa and renal involvement / 中华内科杂志

Objective@#To investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement.@*Methods@#PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed.@*Results@#A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 μmol/L) including creatinine>140 μmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66) years, t2=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ2=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7(18.92%), χ2=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ2=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ2=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05).@*Conclusion@#Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.

Clinical characteristics of 57 patients with polyarteritis nodosa and renal involvement / 中华内科杂志

Objective To investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement. Methods PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed. Results A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 μmol/L) including creatinine>140 μmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66)years, t2=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ2=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7 (18.92%), χ2=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ2=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ2=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05). Conclusion Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.

Clinical characteristics of gastrointestinal involvement in polyarteritis nodosa / 中华风湿病学杂志

Objective To investigate the clinical characteristics of gastrointestinal involvement in polyarteritis nodosa (PAN),and to improve the understanding of the disease.Methods PAN patients hospitalized in Peking Union Medical College Hospital from March 2002 to September 2016 were enrolled in this study,and were divided into gastrointestinal involvement group and non-gastrointestinal involvement group according to clinical manifestations and imaging findings.Data on clinical features,treatments and outcome were recorded.t test,chi-square test were used for statistical analysis.Results A total of 117 patients with PAN were hospitalized in the past 14 years.The prevalence of gastrointestinal involvement was 38％(44 cases).There was no significant difference in age and sex between the two groups (P＞0.05).Abdominal pain (29 cases,66％) was the most frequent manifestation,then gastrointestinal bleeding (10 cases,23％),splenic infarction (3 cases,7％),gastrointestinal ulcers (2 cases,5％),intestinal obstruction or diarrhea (each 2 cases,5％),and vomiting (1 case,2％).Patients with gastrointestinal involvement had more frequent fatigue (27％ vs 11％;x2=5.156,P=0.023),increased diastolic pressure (55％ vs 34％;x2=4.647,P=0.031),renal (34％ vs 18％;x2=3.998,P=0.046) and cardiac (25％ vs 8％;x2=6.225,P=0.013) involvements.ESR in the gastrointestinal involvement group was significantly higher (75％ vs 56％;x2=4.190,P=0.041).The average follow-up time was 315.8 (20.3,441.3) days,the relapse rate was higher in the gastrointestinal involvement group (23％ vs 8％;x2=4.895,P=0.027).The incidence of death or the irreversible organ injury was higher in the gastrointestinal involvement group (27％ vs 11％,x2=5.156,P=0.023).Conclusion Gastrointestinal invol-vement in poly-arteritis nodosa is common and its condition is severe.The incidence of relapse and death or irreversible organ injury is high.

Panniculitides of particular interest to the rheumatologist

Abstract The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous fat with potentially-shared clinical and histopathological features. Clinically, most panniculitides present as red edematous nodules or plaques. Therefore, in addition to a detailed clinical history, a large scalpel biopsy of a recent-stage lesion with adequate representation of the subcutaneous tissue is essential to specific diagnosis and appropriate clinical management. Herein we review the panniculitides of particular interest to the rheumatologist.

Orchitis: An unusual presentation of polyarteritis nodosa

Polyarteritis nodosa (PAN), a systemic necrotizing vasculitis with multiorgan development, is generally restricted to the medium-sized muscular arteries. The varied initial clinical presentations of PAN can lead to a delayed diagnosis. We present the case of a middle-aged male patient who presented with an acute onset right-sided testicular pain as the initial clinical symptom with ischemic changes on ultrasonogram, thereby requiring orchiectomy. This was reported to be a case of tubercular epididymo-orchitis. On review, the biopsy revealed features of necrotizing arteritis as seen in PAN with fibrinoid necrosis and giant cells, thus highlighting the fact that vasculitis due to PAN may have a localized presentation at the time of diagnosis. PAN should be distinguished from other causes of epididymo-orchitis and other vasculitis lesions, the most common being tubercular etiology in the Asian population.

Vasculitic Neuropathy in Cutaneous Polyarteritis Nodosa

No abstract available.

Successful Treatment of Refractory Cutaneous Polyarteritis Nodosa with Adalimumab

Cutaneous polyarteritis nodosa (CPAN) is a form of necrotizing vasculitis of the medium and small-sized arteries. The condition is limited to the skin and there is a lack of visceral involvement. Treatment with systemic glucocorticoids alone or in combination with azathioprine, methotrexate or cyclophosphamide, depending on the disease severity, has been shown to be effective. This paper reports the clinical case of a 53-year-old female patient with CPAN refractory to treatment with high dose glucocorticoid, methotrexate, azathioprine, and cyclophosphamide, who was treated successfully with anti-tumor necrosis factor-α therapy (adalimumab).