Vasculitis reumatoide: a propósito de dos casos / Rheumatoid vasculitis: about two cases

Resumen La vasculitis reumatoide constituye una complicación inusual, pero severa, de la artritis reumatoide, caracterizada por un proceso inflamatorio que compromete vasos sanguíneos de pequeño y mediano calibre, asociada o no a manifestaciones extraarticulares. Algunos factores predisponentes descritos incluyen ciertos haplotipos de antígenos leucocitarios humanos, fumado y enfermedad de larga data. Una disminución en la incidencia ha sido notada desde los años 90 y confirmada en estudios recientes, asociada en particular a un diagnóstico precoz de artritis reumatoide y estrategias terapéuticas que incluyen un aumento en la prescripción de metotrexate. La vasculitis reumatoide se asocia a tasas de mortalidad hasta del 40% a los 5 años y morbilidad importante. Se presentan dos pacientes con diagnóstico previo de artritis reumatoide, con un cuadro crónico de úlceras y disestesias en miembros inferiores, asociado a síndrome anémico. Los estudios histológicos revelaron vasculitis de mediano vaso compatible con vasculitis reumatoide. Se inició manejo con inmunosupresores más esteroides, con resolución del cuadro clínico inicial.

Abstract Rheumatoid vasculitis remains a rare but serious complication of rheumatoid arthritis characterized by an inflammatory process that primarily affects small to medium-sized blood vessels, it can be associated with other extra-articular manifestations. A number of predictor or predisposing factors including certain human leukocyte antigen haplotypes, smoking and long-standing disease. A declining trend in the incidence of rheumatoid vasculitis has been noted since the 1990s and reconfirmed in recent studies, related to early diagnosis of rheumatoid arthritis, and widespread use of methotrexate. Rheumatoid vasculitis is associated with high rates of premature mortality with up to 40% of patients dying by 5 years, as well as a significant morbidity. We present two patients with diagnosis of rheumatoid arthritis with a chronic condition of ulcers and dysesthesias on legs associated with anemic syndrome; the histological study revealed medium size vasculitis. Treatment with inmunosuppresants and steroids was given with resolution of symptoms.

Detrimental Effects of Endovascular Intervention in Active Rheumatoid Vasculitis

Balloon angioplasty can cause shear stress and tear of the vascular endothelium during mechanical dilatation, leading to increased inflammation and coagulation reactions of the vascular endothelium. Herein, a worst case of active rheumatoid vasculitis is described, where due to progressing ischemic necrosis of the leg, endovascular intervention was unavoidably performed in the presence of active rheumatoid vasculitis. After percutaneous balloon angioplasty, the patient developed recurrent thrombotic occlusion of the leg arteries, and finally, limb amputation resulted in despite vigorous treatment including medication, immunosuppression, catheter-directed thrombolysis, and post-thrombolysis anticoagulation. This case report indicates that endovascular intervention may be detrimental to the active rheumatoid vasculitis. Until the development of treatment guideline to prevent or control inflammatory reaction, endovascular intervention for the active rheumatoid vasculitis may not be appropriate as a first line therapy even though there is progressing ischemic necrosis.

A Case of Rheumatoid Vasculitis Involving Hepatic Artery in Early Rheumatoid Arthritis

Rheumatoid vasculitis is a rare, but most serious extra-articular complications of long-standing, seropositive rheumatoid arthritis (RA). Vasculitis of hepatic artery is an extremely rare but severe manifestation of rheumatoid vasculitis. A 72-year-old woman who presented with polyarthralgia for 2 months was diagnosed with early RA. Since she had manifestations of livedo reticularis, and liver dysfunction which was atypical for RA patients, a percutaneous needle liver biopsy was performed revealing arteritis of a medium-sized hepatic artery. Extensive investigations did not reveal evidences of other systemic causes such as malignancy or systemic vasculitis. The patient was diagnosed with rheumatoid vasculitis involving hepatic arteries based on Bacon and Scott criteria for rheumatoid vasculitis. With high dose corticosteroid and cyclophosphamide induction and methotrexate and tacrolimus maintenance treatment, she was successfully recovered. Association of rheumatoid vasculitis at very early stages of the disease may represent an early aggressive form of RA.

Vasculite reumatoide - Relato de caso / Rheumatoid Vasculitis - Case Report

Resumo A artrite reumatoide (AR) é uma doença crônica autoimune inflamatória sistêmica e sua principal manifestação é a sinovite persistente, que compromete articulações periféricas de forma simétrica. Apesar do seu potencial destrutivo, a evolução da AR é muito variável. Alguns pacientes podem ter apenas um processo de curta duração oligoarticular com lesão mínima, enquanto outros sofrem uma poliartrite progressiva e contínua e evoluem com acometimento de outros órgãos e sistemas, como pele, coração, pulmões, músculos e mais raramente vasos sanguíneos, que leva à vasculite reumatoide. O objetivo deste estudo foi descrever um caso de vasculite reumatoide, uma condição rara e grave.

Abstract Rheumatoid arthritis (RA) is a chronic systemic inflammatory autoimmune disease and its main manifestation is persistent synovitis affecting peripheral joints symmetrically, In spite of its destructive potential, the evolution of RA is highly variable. Some patients may have only a short-term process oligoarticular with minimum lesion, while others suffer a polyarthritis evolving with progressive and continuous involvement of other organ systems such as skin, heart, lungs, muscles and blood vessels rarely leading to rheumatoid vasculitis. The aim of this study was to describe a case of rheumatoid vasculitis a rare and severe condition.

The role of KIR2DL3/HLA-C*0802 in Brazilian patients with rheumatoid vasculitis

OBJECTIVES: Rheumatoid arthritis is a polygenically controlled systemic autoimmune disease. Rheumatoid vasculitis is an important extra-articular phenotype of rheumatoid arthritis that can result in deep cutaneous ulcers. The objective of this study was to establish a correlation between the frequency of major histocompatibility complex class I/II alleles and killer immunoglobulin-like receptor genotypes in patients with cutaneous rheumatoid vasculitis. METHODS: Using the Scott & Bacon 1984 criteria to diagnose rheumatoid vasculitis and after excluding any other causes such as diabetes, atherosclerosis, adverse drug reactions, infection, and smoking, patients who met the criteria were selected. All of the selected rheumatoid vasculitis patients presented deep cutaneous ulcers. Identification of the major histocompatibility complex class I/II and killer immunoglobulin-like receptor genotypes was performed by polymerase chain reaction assays of samples collected from the 23 rheumatoid vasculitis patients as well as from 80 controls (40 non-rheumatoid vasculitis RA control patients and 40 healthy volunteers). RESULTS: An association between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and cutaneous lesions in rheumatoid vasculitis patients and a correlation between the inhibitor KIR2DL3 and the HLA-C*0802 ligand in rheumatoid vasculitis patients were found. CONCLUSION: An association was found between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and the development of cutaneous lesions in rheumatoid vasculitis patients. Additionally, the HLA-C*0802 ligand protects these individuals from developing cutaneous lesions. .

Associação do HLA-DRB5*01 com proteção contra manifestação cutânea da vasculite reumatoide em pacientes brasileiros / Association of HLA-DRB5*01 with protection against cutaneous manifestations of rheumatoid vasculitis in Brazilian patients

OBJETIVO: Avaliar a frequência do HLA classes I e II e sua associação com a manifestação cutânea da vasculite reumatoide (VR) em pacientes brasileiros. PACIENTES E MÉTODOS: Durante um ano foram selecionados 130 pacientes com artrite reumatoide (AR), classificados de acordo com os critérios do American College of Rheumatology de 1987. Os pacientes foram submetidos a um questionário clínico e laboratorial para exclusão de outras causas de vasculopatia cutânea (neoplasia, infecções, uso de drogas ilícitas, diabetes mellitus e tabagismo). Setenta e três foram excluídos por apresentarem algum fator de risco para outras causas de vasculopatias. Cinquenta e sete foram incluídos no estudo sem fator de risco para outras causas de vasculopatia, dos quais 17 apresentavam VR de acordo com os critérios de 1984 de Scott e Bacon. Foram analisados dados demográficos, tempo de diagnóstico de AR, atividade da doença (DAS28) e presença de fator reumatoide e de anticorpos antipeptídeo citrulinado cíclico. Alelos HLA foram tipificados usando-se a reação em cadeia da polimerase-DNA amplificada por hibridização de baixa resolução com sequências específicas de sondas. RESULTADOS: A comparação entre os 40 pacientes sem VR e os 17 pacientes com VR demonstrou uma frequência aumentada do HLA-B*14 (Pc = 0,168) e do HLA-Cw*08 (Pc = 0,084) em pacientes com VR, e uma frequência aumentada do HLA-DRB5*01 (Pc = 0,048) em pacientes sem VR. CONCLUSÃO: O HLA-DRB5*01 pode conferir proteção contra essa manifestação extra-articular da AR.

OBJECTIVE: To evaluate the frequency of HLA classes I and II and their association with the cutaneous manifestation of rheumatoid vasculitis (RV) in Brazilian patients. PATIENTS AND METHODS: During one year we selected 130 patients with rheumatoid arthritis (RA) classified according to the American College of Rheumatology, 1987. All patients underwent a clinical and laboratory questionnaire to exclude other causes of cutaneous vasculopathy (neoplasia, infections, illicit drug use, diabetes mellitus, and tobaccoism). Seventy-three patients with any risk factor for other causes of vasculopathy were excluded. Fifty-seven without risk factors for other causes of vasculopathy were included in the study, 17 with RV according to Scott and Bacon's criteria, 1984. Demographic data, time of RA diagnosis, disease activity (DAS28), presence of rheumatoid factor, and anti-cyclic citrullinated peptide antibodies were analyzed. The HLA alleles were typed using the DNA-amplified polymerase chain reaction with low-resolution hybridization and sequence-specific primers. RESULTS: The comparison between the 40 patients without RV and the 17 patients with RV showed an increased frequency of HLA-B*14 (Pc = 0.168) and HLA-Cw*08 (Pc = 0.084) in patients with RV and an increased frequency of HLA-DRB5*01 (Pc = 0.048) in patients without RV. CONCLUSION: The HLA-DRB5*01 may confer protection against that extra-articular manifestation of RA.

A Case of Acute Myocardial Infarction and Multiorgan Involvement Secondary to Rheumatoid Vasculitis

Rheumatoid arthritis patients have an increased risk of subclinical cardiovascular disease, and they also have a high prevalence of carotid disease and peripheral arterial disease as a form of vasculitis. Rheumatoid arthritis patients have an increased cardiovascular mortality rate and an increased premature death rate, and they have a higher incidence of atherosclerosis. Myocardial infarction due to vasculitis is a rare complication for patients with rheumatoid vasculitis. We report here on a case of a patient with multiorgan involvement who developed myocardial infarction, right carotid artery occlusion and left renal artery occlusion secondary to his rheumatoid vasculitis.

Angiotensin Converting Enzyme Insertion/Deletion Polymorphism in Korean Patients with Rheumatoid Vasculitis / 대한류마티스학회지

OBJECTIVE: Rheumatoid arthritis has various extra-articular manifestations including rheumatoid vasculitis. Angiotensin converting enzyme (ACE) gene shows insertion/deletion polymorphism and has II, ID, DD genotypes. ACE gene is related with vasoconstriction and endothelial dysfunction in cardiovascular disease. This study was undertaken to determine the association between ACE gene polymorphism and rheumatoid vasculitis. METHODS: Twenty-nine patients were collected as rheumatoid vasculitis group. DNA was isolated from blood samples collected from 114 Korean rheumatoid arthritis patients meeting American College of rheumatology 1987 revised criteria, and 114 healthy control group. Genotyping for the angiotensin converting enzyme gene insertion/deletion polymorphism was performed by polymerase chain reaction method. RESULTS: As vasculitis manifestation, 15 patients showed neuropathy, 13 showed scleritis, 3 showed skin rash. In rheumatoid vasculitis group, II, ID and DD polymorphism was seen in 8 (27.6%), 15 (51.7%), 6 (20.7%) patients respectively and 39 (34.2%), 57 (50.0%), and 18 (15.8%) in normal controls. There was no skewing of ACE I/D polymorphism in compared with normal group. In rheumatoid arthritis control group, II, ID and DD polymorphism was seen in 37 (32.5%), 64 (56.1%), and 13 (11.4%) patients. Among rheumatoid arthritis patient, there was no significant difference between patient with vasculitis and without vasculitis. CONCLUSION: Our results showed that genetic polymorphisms of angiotensin converting enzyme insertion/deletion gene has no association with the susceptibility to rheumatoid vasculitis.

A Case of Rheumatoid Vasculitis / 대한피부과학회지

Rheumatoid vasculitis usually occurs in patients with long standing, seropositive, erosive rheumatoid arthritis. It involves typically small to medium-sized vessels and is associated with peripheral neuropathy, digital gangrene, nail fold infarcts, livedo reticularis, and palpable purpura. Histologic examination of skin biopsy specimens usually shows leukocytoclastic vasculitis. We report a case of rheumatoid vasculitis developed on both extremities in a 63-year-old female with rheumatoid arthritis for 15 years.

Rheumatoid vasculitis manifesting as ischemic colitis / 대한내과학회지

Rheumatoid vasculitis is a necrotizing arteritis of unknown causes that affect a subset of patients with rheumatoid arthritis. Vasculitis associated with rheumatoid arthritis was first recognized in 1898. It was described frequently in the 1940s through 1960s but is now rarely diagnosed. Typical clinical features include constitutional symptoms, mononeuritis multiplex, skin infarction, ulceration, peripheral gangrene and visceral infarction. Gastrointestinal involvement occurs in 10 to 38% of cases of rheumatoid vasculitis and may include bowel infarction, ulceration, perforation, colitis, stricture, or bleeding. Ischemia of the intestine is the end result of interruption or reduction of its blood supply. However, the clinical manifestations of intestinal ischemia range from mild chronic symptoms to a catastrophic acute episode, depending on the vascular supply involved, the extent of the occlusion or ischemia, and the rapidity of the process. Physicians caring for patients with rheumatoid arthritis should be aware that intestinal ischemia or infarction may occur without overt clinical evidence of systemic rheumatoid vasculitis. Herein we describe a case of rheumatoid vasculitis manifesting as ischemic colitis which was intractable to medical therapy and led to subtotal colectomy.

3 Cases of Mononeuropathy Multiplex Associated with Systematic Vasculitis

Peripheral nervous system involvement is common in systemic vasculitis, occurring most frequently in the polyarteritis nodosa (PAN) group of disorders and in rheumatoid vasculitis. Within the polyarteritis nodosa group of systemic necrotizing vasculitides, three subgroups have been described: classic polyarteritis nodosa, Churg-Strauss syndrome, and an overlap syndrome. Three patients with evidence of systemic vasculitis and peripheral neuropathy were clinically and electrophysiologically investigated. All cases presented clinically with mononeuropathy multiples considered typical pattern of ischemic involvement of the peripheral nerve. The causes included polyarteritis nodosa, its Churg-strauss variant, and the overlap syndrome. Pain and weakness were frequent symptoms. Nerve conduction studies were abnormal In all cases. Necrotizing vasculitis was present as pathologic findings in two cases. All patients were treated with prednisolone alone or in combination with other immunosuppressive agents or with plasmapheresis.

A Case of Sjogren Syndrome Associated with Acroosteolysis / 대한류마티스학회지

Acroosteolysis (AOL) refers to a destructive process involving distal phalangeal shaft while the tuft and base are preserved. It can be a manifestation of various diseases, such as scleroderma, Raynaud's disease, rheumatoid vasculitis, psoriasis, renal osteodystrophy and leprosy. Occupational exposure to polyvinyl chloride polymers, thermal injury, and repetitive mechanical injury can also cause this problem. Although the exact pathogenesis of AOL is uncertain, a unifying theme of vascular and mechanical injury is presented. Recently, we experienced a 32-year-old woman complained of xerophthalmia, zerostomia and polyarthralgia which was diagnosed as Sj6gren syndrome associated with acroosteolysis. After administration of prednisolone, cyclophosphamide and oral bromhexine, subsidence of her symptoms was observed. We report this case with a review of relevant literature.