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.

An update of neurological manifestations of vasculitides and connective tissue diseases: a literature review / Atualização das manifestações neurológicas das vasculitides e das doenças do tecido conjuntivo: revisão de literatura

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.

As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.

La artritis de la poliarteritis nodosa cutánea en niños se relaciona con la infección por estreptococos / Arthritis in cutaneous polyarteritis nodosa and its relationship with streptococcus

La poliarteritis nodosa cutánea (PANC) es una rara vasculitis que afecta los vasos de mediano calibre de la dermis, músculo, nervios periféricos y articulaciones, y que está frecuentemente relacionada con infección por estreptococo beta hemolítico del grupo A (EBHGA). Las características clínicas de la PANC posestreptocócica son fiebre, nódulos subcutáneos dolorosos y artritis. En nuestra cohorte de 12 pacientes, la artritis tuvo lugar con mayor frecuencia en las articulaciones grandes de miembros inferiores y tuvo una duración variable, desde días hasta meses. Este cuadro clínico, observado en niños de edad escolar, simula el de la fiebre reumática aguda, y por lo tanto puede llevar a la formulación de un diagnóstico erróneo. La presencia de nódulos con superficie rojiza, exquisitamente dolorosos, tumefacción subcutánea y muscular difusa y dolorosa, lesiones livedoides, así como la ausencia de compromiso cardíaco son orientadoras hacia el diagnóstico de PANC, que es confirmado ante el hallazgo de vasculitis de vasos medianos en la dermis. Si bien la respuesta a los esteroides sistémicos suele ser excelente, las recaídas son frecuentes y casi invariablemente asociadas a una nueva infección faríngea por EBHGA, por lo que es imperativo el uso profiláctico de penicilina a largo plazo.

Poliarterite nodosa: revisão de literatura a propósito de um caso clínico / Polyarteritis nodosa: literature review related to a case report

A poliarterite nodosa é uma doença rara. Trata-se de uma vasculite sistêmica caracterizada pela presença de um processo inflamatório agudo e necrose fibrinoide das artérias de pequeno e médio calibre. O curso pode ser agudo ou crônico, com grande variabilidade de sinais e sintomas devido aos diversos órgãos que podem ser afetados, preferencialmente o sistema nervoso periférico, rins e pele. Os autores relatam o caso de um paciente com manifestações clínicas e evolução incomum.

Polyarteritis nodosa is a rare disease, being a systemic vasculitis characterized by the presence of an acute inflammatory process and fibrinoid necrosis of small and medium-sized arteries. Its course may be acute or chronic, with large variability of signs and symptoms due to various organs that may be affected, mainly peripheral nervous system, kidneys, and skin. The authors report the case of a patient with unusual clinical manifestations and course.

Vasulitis primarias en la infancia: estudio clínico-epidemiológio / Primary vasculitis in children: a clinical-epidemiology study

Introducción. La clasificación de las vasculitis primarias es dificultosa y su prevalencia en la edad pediátrica, poco conocida. Objetivo. Determinar la frecuencia, los datos epidemiológicos y las características clínicas de las vasculitis primarias en pacientes pediátricos entre mayo de 2000 y mayo de 2008. Diseño. Estudio observacional, retrospectivo y descriptivo. Materiales y métodos. Revisión de la base de datos de las historias clínicas de vasculitis primarias en pacientes pediátricos de 0 a 16 años que cumplían los criterios de inclusión establecidos. Resultados. Se encontraron 47 pacientes, 29 varones y 18 mujeres. La edad promedio de presentación fue de 4 años (rango: 7 meses a 13 años). La vasculitis más frecuente fue la púrpura de Schõnlein-Henoch, con 33 registros (70%), seguida por la enfermedad de Kawasaki 9 (19%), el edema agudo hemorrágico del lactante 3 (6%), la panarteritis nodosa cutánea 1 (2%) y el síndrome de Churg-Strauss 1 (2%). Conclusión. Las vasculitis primarias son poco frecuentes en la edad pediátrica. La púrpura de Schõnlein-Henoch fue la más frecuente en nuestra población. La enfermedad de Kawasaki fue la vasculitis de mayor morbilidad. El edema agudo hemorrágico del lactante se presentó en niños menores de 2 años, fue menos frecuente y con escasa morbilidad. Pudimos también detectar 2 casos de vasculitis menos frecuentes como la panarteritis nodosa cutánea y el síndrome de Churg-Strauss.

Introduction.The classifi cation of primary vasculitis is diffi cult and the prevalence in childhood is unknown. Objective. To determine the frequency, epidemiological data and the clinical features of the primary vasculitis in pediatric patients seen in our Hospital since May 2000 to may 2008. Design. Observational, retrospective and descriptive study. Materials and methods. We reviewed the data base of medical records of the pediatric patients with primary vasculitis diagnose. We included patients from 0 to 16 years old that met the established inclusion criteria. Results. Forty-seven patients were found, 29 were boys and 18 girls. The average age of onset was 4 years old (range: 7 months to 13 years old). The most frequent vasculitis was the Henoch Schönlein purpura with 33 cases(70%), followed by the Kawasaki disease with 9 patients (19%); the acute hemorrhagic edema of young children with 3 cases (6%) and the cutaneous polyarteritis nodosa and the Churg-Strauss syndrome with 1 case each. Conclusions. Primary vasculitis is not frequent in childhood. The He-noch Schönlein purpura was the most frequent in our patients. The Kawasaki disease was the vasculitis with major morbidity. The acute hemorrhagic edema of infancy was less frequent, showed less morbidity and presented in children younger of two years of age. We had also the opportunity to observed 2 cases of very low-frequency vasculitis such us cutaneous polyarteritis nodosa and Churg-Strauss syndrome.

Alelos HLA-DR em pacientes com poliarterite nodosa e poliangite microscopica / Alleles HLA-DR in patients with polyarteritis nodosa and microscopic polyangiitis

O presente estudo avaliou a freqüência dos alelos HLA-DR em um grupo de 29 pacientes brasileiros caucasóides com PAN ou poliangiíte microscópica e investigou uma possível relação entre os alelos HLA-DR, os índices de atividade e gravidade e as manifestações clínicas de doença. O diagnóstico de cada paciente baseou-se nos critérios do Colégio Americano de Reumatologia (ACR) para a PAN e da Conferência Internacional de Consenso de Chapel Hill (CHCC) para poliangiíte microscópica. A atividade e a gravidade da doença foram mensuradas retrospectivamente, por ocasião do diagnóstico, utilizando-se, respectivamente, o Birmingham Vasculitis Activity Score (BVAS) e o Five-Factors Score (FFS). De acordo com o BVAS, os pacientes foram divididos em dois grupos: aqueles com BVAS < 22 e outro com BVAS ³ 22. De acordo com o FFS, dois grupos foram definidos: 0, quando nenhum fator de pior prognóstico foi notado e ³ 1 quando 1 ou mais fatores estavam presentes. As manifestações clínicas presentes por ocasião do diagnóstico foram avaliadas de acordo com o sistema orgânico acometido. Cinquenta e nove indivíduos caucasóides, saudáveis, da mesma população, formaram o grupo controle. A tipagem dos alelos HLA-DR foi realizada através da técnica de amplificação pela reação em cadeia da polimerase (PCR), utilizando-se sequências específicas de primers DR de baixa resolução. No grupo total de casos, encontrou-se uma maior frequência estatisticamente significativa de HLADRB1* 16 (p=0.023) e DRB4*01 (p=0.048) nos pacientes com BVAS ³ 22. Os pacientes com FFS=0 apresentaram uma maior frequência estatisticamente significativa de HLA-DRB1*03. A frequência de HLA-DRB1*11 e/ou B1*12 (p=0.046), B1*13 (p=0.021) e B3 (p=0.008) foi significativamente maior nos pacientes com envolvimento do trato gastrintestinal...

The aim of this study was to evaluate the frequency and clinical associations of HLA-DR alleles in Brazilian Caucasian patients with polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA). We evaluated 29 Caucasian patients with vasculitis classified as PAN or MPA according to the American College of Rheumatology (ACR) 1990 Criteria and Chapel Hill Consensus Conference (CHCC) nomenclature for vasculitis. HLA-DR alleles were typed using polymerase chain reaction-amplified DNA, hybridized with sequence specific low resolution primers. DNA obtained from 59 Caucasian healthy blood donors were used as control. In order to evaluate if a specific HLA may have influence on the clinical profile of those diseases, we also divided the patients according to Birmingham vasculitis score (BVAS) and Five-Factors Score (FFS) at the time of diagnosis. Increased frequency of HLA-DRB1*16 (p=0.023) and DRB4*01 (p=0.048) was found in patients with higher disease activity at the time of diagnosis (BVAS ³ 22). Patients with less severe disease (FFS=0) had a higher frequency of HLA-DRB1*03 (p=0.011). Patients with gastrointestinal tract involvement had significantly increased frequency of HLA-DRB1*11 or B1*12 (p=0.046), B1*13 (p=0.021) and B3 (p=0.008). In contrast, patients with renal disease, had higher frequency of DRB1*15 or DRB1*16 (p=0.035) and B5 (p=0.035). Our results suggest that HLA-DR alleles may influence PAN and MPA clinical expression and outcome.

Clinical Features and Outcomes of Microscopic Polyangiitis in Korea

Microscopic polyangiitis (MPA) is a systemic vasculitis affecting small vessels. To determine the clinical features and outcomes of MPA in Korean patients, we retrospectively reviewed the medical records of patients diagnosed with MPA at a single medical center in Korea between 1989 and 2006. The 18 patients who met the Chapel Hill criteria for MPA had a mean (+/-SD) age at the time of diagnosis of 62.4+/-12.7 yr. Renal manifestations and general symptoms were the most common features of MPA, with lung involvement also very common. Antineutrophil cytoplasmic antibodies (ANCA) were present in 17 of the 18 patients (94%). Of 17 patients treated with steroids and cyclophosphamide, 11 (65%) had stable or improved course. One patient treated with steroids without cyclophosphamide showed disease progression. Ten of the 18 patients (56%) died at a median follow-up of 8 months. MPA in Korean patients was distinguished by a higher rate of lung involvement, especially alveolar hemorrhage, which was the leading cause of death in our patients. Korean patients were also older at MPA onset and were more likely positive for ANCA. Other overall clinical manifestations did not differ significantly.

Nódulos mamários como manifestação primária de poliarterite nodosa / Breast nodules as a primary manifestation of polyarteritis nodosa

A poliarterite nodosa é uma vasculite necrosante sistêmica caracterizada por processo inflamatório agudo e necrose fibrinóide das artérias de pequeno e médio calibre. É uma doença rara, com prevalência na população geral de cerca de 6 por 100.000 pessoas, e duas vezes mais freqüente em homens. O quadro clínico é variável e constituído usualmente por sintomas gerais (febre, astenia, emagrecimento) e sintomatologia relacionada com envolvimento multiorgânico (articular, neurológico, cutâneo, digestivo, renal, cardíaco e hipertensão arterial). Outros órgãos também podem estar envolvidos mais raramente, como: testículo, útero, mama e retina. Relatamos o caso de uma paciente do sexo feminino com diagnóstico de PAN firmado após biópsia de nódulo mamário. O envolvimento mamário é uma forma não usual de manifestação de PAN, sendo descritos na literatura cerca de dez casos, em sua maioria lesões mamárias como achados isolados e com prognóstico favorável.

Polyarteritis nodosa is a systemic necrotizing vasculitis chacterized by an acute inflammatory process and fibrinoid necrosis of small and medium vessels. It is a rare disease with prevalence in the general population of 6 per 100,000 people. It is more common in men with a male/female ratio of 2:1. The clinical features are variable but most patients present with generalized symptoms including fever, fatigue and weight loss and/or the symptoms can be related to multi-organ involvement (skin, gastrointestinal tract, joints, nervous system, kidneys, cardiovascular system). Rarely, other organs can be compromised such as breasts, testicles, uterus, and retina. In this case report, a female patient had the diagnosis of polyarteritis nodosa (PAN) made after a breast nodule biopsy. Localized involvement of the breast is unusual manifestation of PAN. To date, 10 cases have been reported, in most cases breast lesions were an isolated finding, and the prognosis was favourable.

Childhood polyarteritis nodosa: a clinical diagnosis.

A six-year old boy presented with dry gangrene of toes and fingers with hypertension with no other systemic abnormalities. He had persistently high inflammatory parameters, was diagnosed as childhood classic polyarteritis nodosa and showed improvement with immunosuppressants along with antihypertensives. Toe gangrene required amputation in view of superadded infection.

Poliarteritis nodosa cutánea posestreptocócica: un simulador de la fiebre reumática / Pos-streptoccal cutaneous polyarteritis nodosa: a mimic of rheumatic fever

RESUMEN Introducción. La poliarteritis nodosa cutánea es una enfermedad inflamatoria que compromete vasos medianos de piel y, en ocasiones, de nervios periféricos y músculos. La frecuente presencia de artritis puede originar errores diagnósticos. El objetivo fue describir los hallazgos clínicos en pacientes con esta enfermedad.Población, material y métodos. Se trató de un estudio descriptivo. Se incluyeron 10 niños con diagnóstico de poliarteritis nodosa cutánea y evidencias de infección reciente por estreptococo betahemolítico del grupo A. Los datos se recolectaron retrospectivamente.Resultados. Todos los pacientes presentaban fiebre,púrpura y nódulos subcutáneos en el debut de la enfermedad. Nueve tuvieron artritis. Las articulaciones afectadas fueron predominantemente grandes,de miembros inferiores y la duración de la inflamación articular varió entre 10 y 90 días. Seis pacientes mostraron un patrón aditivo de afección articular y en tres fue migratorio. La articulación más frecuentemente afectada fue la rodilla. Se observó leucocitosis y elevación de los reactantes de fase aguda en todos los pacientes. Los nueve niños con artritis cumplían con los criterios diagnósticos de Jones para fiebre reumática. Todos recibieron tratamiento con m menos prednisona y profilaxis con penicilinabenzatínica. Se observaron 17 recaídas en 8 de los pacientes, 12 de ellas relacionadas con nuevas infecciones por estreptococo y fallas en la profilaxis. Conclusiones: La artritis de la poliarteritis nodosa cutánea posestreptocócica puede remedar a la de la fiebre reumática. La erupción purpúrica y nódulos cutáneos rojizos y dolorosos orienta fuertemente hacia el diagnóstico, que se confirma por el hallazgo de inflamación de vasos medianos en la dermis

An unusual presentation of polyarteritis nodosa: A case report / Una manifestación poco usual de la poliarteritis nodosa: Reporte de un caso

Polyarteritis nodosa with gallbladder involvement is a rare condition. Autosomal dominant polycystic kidney disease is also a rare condition and rarely complicated. We describe an extremely rare case of Polyarteritis nodosa, involving gallblader and ureter without obstruction, in a patient with autosomal dominant polycystic kidney disease. To the best of the authors' knowledge, such a case has not been reported previously.

La periarteritis nodosa con compromiso de la vesícula es una condición rara. La enfermedad poliquística renal autosómica dominante es también una condición rara y raramente complicada. Describimos un caso extremadamente raro de poliarteritis nodosa, con compromiso de la vesícula y el uréter sin obstrucción, en un caso de enfermedad poliquística renal autosómica dominante. Al leal saber y entender de los autores, no ha sido reportado antes un caso como éste.

[Periarteritis nodosa: experience of medecine interne]

Periartritis nodosa is a rare necrotic type of systemic vasculitis [angiitis] characterized by a multivisceral affection. Diagnosis is essentially histological. The prognosis has been improved, as a result of the therapeutic progress and the early management of this disease in the specialized centers. We report the experience of the internal medicine unit in the management of this disease depending on the new therapeutic strategies. It is a retrospective study carried out in the internal medicine unit of Ibn Sina hospital of Rabat through a period of 16 years between 1987 and 2003 responding to the clinico-biological criteria of the American College of Rheumatology [ACR] 1990- A series of twenty four cases 12 male and 12 female aged between 17 and 74 years with an age average of 45 years. Several parameters were evaluated: clinical manifistations, biological! [sedimentation rate-blood picture creatininemia], bacteriologically [B-C] serology], electromyographical and mainly neuromascular and or cutenous biopsy which assures the certitude of the diagnosis and also therapeutically [evaluation of the treatment]. Neuromuscular and or cutaneous biopsy showed lesions in favour of periarteritis nodosa in 12 patients, the other patients diagnosis was made according to the ACR criteria. As for the treatment, all patients were put under corticosteroid therapy, four severe form were treated by an association of cyclophosphamide and corticoides. Evolution was favourable in 15 patients, with recurrence in 3 cases, death of one case and 5 other patients were lost of sight. The prognosis of periartitis nodosa can be radically modified by the early diagnosis in the specialized centers

Hemobilia as presenting manifestation of polyarteritis nodosa.

Polyarteritis nodosa (PAN) is a multisystem necrotizing inflammatory vasculitis of medium-sized muscular arteries. Hepatobiliary complications secondary to thrombosis or aneurysm formation in PAN are rare and are associated with poor outcome. We describe an 18-year-old man with PAN who presented with hemobilia secondary to rupture of a hepatic artery aneurysm, which was successfully managed with coil embolization followed by immunosuppressive therapy.

Isolated necrotizing vasculitis of gallbladder: a report of two cases and review of literature.

We report two cases of isolated necrotizing vasculitis of gallbladder without evidence of systemic involvement which was histologically indistinguishable from classic polyarteritis nodosa (PAN), displaying vascular lesions in different stages of progression like those of PAN. The literature has been reviewed in order to evaluate the significance and possible aetio-pathogenesis of gallbladder (GB) vasculitis. Localized PAN of gallbladder is a rare incidental postoperative finding, the serological work-up for autoantibodies may be negative and the disease is likely to remain self-limited.

Clinical approach to vasculitides.

The term vasculitis refers to inflammation and necrosis of blood vessels. The vasculitides are best classified according to the size of the involved vessels into large, medium and small vessel vasculitis. As a group the disorders are not uncommon even in children but the diagnosis of a given condition can often get delayed, or ever be entirely missed, because of multi-system involvement and the consequent protean clinical manifestations. Kawasaki Disease and Henoch-Schonlein Purpura are the commonest vasculitides seen in children. Early diagnosis and prompt treatment can go a long way in decreasing the morbidity and mortality associated with these disorders.

Hepatitis B-associated Polyarteritis Nodosa and Hypertensive Encephalopathy

The case of a two-year old boy with hypertensive encephalopathy to Hepatitis B-associated polyarteritis nodosa is presented. Polyarteritis nodosa is a rare condition in children and its association with Hepatitis B viral infection is also rare

Avances en el diagnóstico de las vasculitis sistémicas / Recent progress in the diagnosis of systemic vasculitis

The clinical picture of primary systemic vasculitis, that cause inflammation and necrosis of vessel walls, depend on the type, size and location of involved vessels. Frequently, their clinical presentation does not reflect the specific type of vasculitis and its prognosis. Moreover, the correct diagnosis, early and adequate treatment have an important prognostic value. Recently, new diagnostic methods for systemic vasculitis have been devised. This article critically reviews and provides information for the rational use of these new imaging techniques and laboratory procedures for the diagnosis and follow up of systemic vasculitis