Vasculitis reumatoidea como manifestación extraarticular en dos casos / Rheumatoid vasculitis as an extra articular manifestation in two cases

La vasculitis reumatoidea es un proceso inflamatorio poco frecuente que confiere una morbilidad y mortalidad significativa en pacientes con artritis reumatoidea (AR). Presenta una incidencia de 0,7 a 5,4% de los casos de AR y hasta un 40% de mortalidad a 5 años, siendo la manifestación extraarticular de mayor gravedad1,2. Se caracteriza por el desarrollo de vasculitis necrotizante; ocurre típicamente en pacientes masculinos con AR seropositiva de larga data, nodular y erosiva, tabaquistas y, en general, tiene mal pronóstico. Se presenta una serie de casos, paciente masculino y femenino con AR y manifestaciones extraarticulares, necrosis digital y compromiso multiorgánico, interpretados como cuadros de vasculitis reumatoidea, realizando tratamiento de referencia con inmunosupresores.

Rheumatoid vasculitis is a rare inflammatory process that confers significant morbidity and mortality in patients with rheumatoid arthritis (RA); it has an incidence of 0.7 to 5.4% of RA cases and up to 40% mortality at five years, making it the most serious of all the extra-articular manifestations of RA1,2. It is characterized by the development of necrotizing vasculitis, it typically occurs in male patients with long-standing seropositive RA, erosive nodular, smokers and generally has a poor prognosis. A series of cases is presented, male and female with RA and extra-articular manifestations, digital necrosis and multiorgan system involvement, interpreted as rheumatoid vasculitis, undergoing treatment with immunosuppressants.

Molecular confirmation of ovine herpesvirus 2-induced malignant catarrhal fever lesions in cattle from Rio Grande do Norte, Brazil / Diagnóstico molecular de herpesvírus ovino tipo 2 em surto de febre catarral malígna em bovinos do Rio Grande do Norte

Molecular findings that confirmed the participation of ovine herpesvirus 2 (OVH-2) in the lesions that were consistent with those observed in malignant catarrhal fever of cattle are described. Three mixed-breed cattle from Rio Grande do Norte state demonstrated clinical manifestations that included mucopurulent nasal discharge, corneal opacity and motor incoordination. Routine necropsy examination demonstrated ulcerations and hemorrhage of the oral cavity, corneal opacity, and lymph node enlargement. Significant histopathological findings included widespread necrotizing vasculitis, non-suppurative meningoencephalitis, lymphocytic interstitial nephritis and hepatitis, and thrombosis. PCR assay performed on DNA extracted from kidney and mesenteric lymph node of one animal amplified a product of 423 base pairs corresponding to a target sequence within the ovine herpesvirus 2 (OVH-2) tegument protein gene. Direct sequencing of the PCR products, from extracted DNA of the kidney and mesenteric lymph node of one cow, amplified the partial nucleotide sequences (423 base pairs) of OVH-2 tegument protein gene. Blast analysis confirmed that these sequences have 98-100% identity with similar OVH-2 sequences deposited in GenBank. Phylogenetic analyses, based on the deduced amino acid sequences, demonstrated that the strain of OVH-2 circulating in ruminants from the Brazilian states of Rio Grande do Norte and Minas Gerais are similar to that identified in other geographical locations. These findings confirmed the active participation of OVH-2 in the classical manifestations of sheep associated malignant catarrhal fever.

Os achados moleculares confirmaram a participação do herpesvírus ovino tipo 2 (OVH-2) nas lesões observadas em um surto de febre catarral malígna em bovinos. Três bovinos oriundos de propriedade rural de Mossoró, Rio Grande do Norte apresentaram manifestações clínicas, que incluíram secreção nasal mucopurulenta, opacidade da córnea e incoordenação motora. A necropsia revelou ulcerações e hemorragias da cavidade oral, opacidade da córnea e linfonodomegalia. Os achados histopatológicos significativos incluíam vasculite necrosante generalizada, meningoencefalite não supurativa, nefrite intersticial linfocítica, hepatite linfocítica e trombose. A PCR, realizada a partir de DNA extraído do rim e do linfonodo mesentérico de um dos animais, amplificou um produto com 423 pares de base do gene da proteína do tegumento do herpesvírus ovino 2 (OVH-2). O sequenciamento direto dos produtos da PCR e a análise pelo Blast demonstraram que o produto amplificado apresentava 98-100% de identidade com sequências do OVH-2 depositadas no GenBank. As análises filogenéticas, baseadas nas sequências deduzidas de aminoácidos demonstraram que a cepa de OVH-2 circulando em ruminantes nos estados de Rio Grande do Norte e Minas Gerais são semelhantes àquelas identificadas em outras regiões geográficas. Esses achados confirmam a participação ativa de OVH-2 nas manifestações clássicas de febre catarral maligna em ovinos.

Perirenal hematoma and involvement of the temporal artery in a patient with polyarteritis nodosa (PAN) / Hematoma perirrenal e envolvimento da artéria temporal em paciente com poliarterite nodosa (PAN)

Descrevemos o caso de um paciente masculino, 48 anos, com poliartralgias e mialgias migratórias, associadas a febre e emagrecimento com dois meses de evolução; um episódio de dor testicular bilateral; e cefaleia unilateral pulsátil com espessamento de artéria temporal esquerda sugerindo arterite temporal. Na evolução, o paciente apresentou hematoma perirrenal esquerdo espontâneo, infartos esplênicos e insuficiência renal aguda oligúrica. Foi tratado com prednisona e ciclofosfamida. Foram realizadas biópsias de artéria temporal esquerda e músculo quadríceps. Os achados clínicos, laboratoriais, radiológicos e intercorrências levaram ao diagnóstico de poliarterite nodosa (PAN).

We report the case of a 48-year-old male with a 2-month history of migratory polyarthralgia, and myalgia associated with fever and weight loss; one episode of bilateral testicular pain; and unilateral pulsatile headache with thickening of the left temporal artery suggestive of temporal arteritis. The patient evolved with spontaneous left perirenal hematoma, splenic infarcts, and oliguric acute renal failure. Treatment included prednisone and cyclophosphamide. The left temporal artery and the quadriceps muscle were biopsied. Clinical, laboratorial, and radiological findings, as well as the intercurrences, led to the diagnosis of polyarteritis nodosa.

A Case of Fatal Wegener's Granulomatosis without Anti-neutrophil Cytoplasmic Antibody (ANCA) / 대한피부과학회지

Wegener's granulomatosis (WG) is a systemic necrotizing vasculitis belonging to the small- to medium-sized vessel. A 46-year-old male presented with erythematous hard plaque on chest, calf and multiple subcutaneous nodules on buttock, upper arm. Incisional biopsy was done and the histopathologic finding revealed the features of necrotizing vasculitis with focal granulomatous inflammation in subcutaneous tissue. PNS MRI showed abnormal meningeal enhancement and lung HRCT imaging showed mild infiltration. Inspite of severe systemic involvement, serum tests about ANCA showed negative result. He was treated with systemic corticosteroid and cyclophosphamide pulse therapy. Initially his condition showed improvement but later the disease worsened.

Three Cases with Cutaneous Polyarteritis Nodosa / 대한류마티스학회지

Systemic polyarteritis nodosa is a necrotizing vasculitis that involves small and medium-sized muscular arteries in the multiple organ systems, whereas cutaneous polyarteritis nodosa (CPAN) is a localized disease characterized by necrotizing vasculitis of small and medium-sized arteries in the skin without life-threatening organ involvement. CPAN is usually limited to skin, muscle, and joints. It is chronic but takes a benign course. The most common cutaneous manifestations include nodules, ulcers and a livedo reticularis pattern on the extremities. We describe three cases with CPAN showing necrotizing vasculitis on the skin without systemic symptoms or visceral involvement. In Korea, most previously published cases with CPAN have shown a good response to a short term of corticosteroids or colchicine administration. However, two of our three patients required another immunosuppressive agent in addition to corticosteroids to manage their intractable skin lesions, and the remaining one showed a good response to a short course of prednisolone. In addition, there was one patient with CPAN having hepatitis B surface antigen, which might be associated with his refractory skin disease.

A Case of Mumps Virus Infection Associated with Severe Myositis / 감염

Myositis caused by mumps virus is very rare. Mumps virus has been suggested as a causative agent of inclusion body myositis, but there has been no definite evidence. We experienced a case of severe myositis associated with mumps virus infection. A 21-year old man was admitted because of myalgia, headache, fever, and chill for 2 months. The cerebrospinal fluid analysis performed at a local clinic showed findings compatible with viral meningitis. His blood chemistry results revealed elevated levels of serum creatine kinase, lactate dehydrogenase, and serum myoglobin. On the 5th day of admission, the patient showed pain and swelling of parotid gland and scrotum. Electromyography was compatible with inflammatory myopathy. Muscle biopsy of his calf muscle revealed necrotizing (leukocytoclastic) vasculitis and multifocal myolysis with multinucleation. We suspected mumps virus infection because of his symptoms of meningitis, epididymo-orchitis and parotitis. Mumps virus was isolated in throat culture. The past medical history of the patient's mumps virus vaccination was unclear. After 2 weeks of supportive treatment, the patient's condition was improved.

Electrophysiologic Characteristics in Systemic Necrotizing Vasculitis Patients with Peripheral Neuropathy

OBJECTIVE: To evaluate the clinical findings and the results of an electrophysiological study in seven patients with a systemic necrotizing vasculitis and neuropathy. METHOD: Clinical and electrophysiological studies were performed in seven patients of whom three had a polyarteritis nodosa, other three a Churg-Strauss syndrome, and one an overlapping syndrome. RESULTS: In the electrophysiological study, five patients presented an overlapping multiple mononeuropathy, one a multiple mononeuropathy and the other one a distal symmetric polyneuropathy. The neuropathies were mainly axonopathies with a decreased amplitude of compound motor action potential and sensory nerve action potential; however one patient showed the conduction blocks in the tested nerves. In electromyography, the most prominent feature was a neurogenic pattern with fibrillation potentials and reduced recruitments. No patient had a myopathic pattern. CONCLUSION: The most prominent features of the systemic necrotizing vasculitis patients with neuropathy were the axonopathic findings in the nerve conduction studies and neurogenic pattern in the electromyographic studies. Electrodiagnostic study was helpful for the diagnosis of vasculitic neuropathy, however further studies would be necessary to clarify the characteristics of the neuropathy of polyarteritis nodosa and the Churg-Strauss syndrome.

A Case of Henoch-Schoenlein Purpura with Necrotizing Vasculitis in the G-I Tract / 대한피부과학회지

Henoch-Schoenlein purpura is a syndrome of acute systemic allergic vasculitis involving the small vessels of skin and multiple organs, characterized by a symmetrical, non-thrombocytopenic, painless purpura, nephritis and gastrointestinal manifestations. Although GI involvement is about 70%, endoscopic and histopathological finding of the GI tract in Henoch-Schoenlein purpura is rarely reported and necrotizing vasculitis in GI tract biopsy has not been reported yet. We report a case of a 16-year-old male patient, who complained of palpable purpura, vomiting and epigastric pain with necrotizing vasculitis of the duodenum on histopathological examination.

Wegener' s Granulomatosis / 대한피부과학회지

Wegener's granulomatosis is a systemic necrotizing vasculitis of unknown cause. The disease is characterized by the involvement of the upper airway, the lung, and the kidney. Skin lesions are frequent and the most common lesion is purpura distributed on the limbs and trunk. A 34-year-old female showed recurrent purpuric macules on the both lower extremities and buttocks. The patient. showed nasal septal perforation with saddle nose deformity and C-ANCA positivity. Histopathologic findings of purpuric lesion revealed the features of necrotizing vasculitis. The histologic specimen from the nasal cavity showed chronic inflammation with granuloma formation and kidney showed focal necrotizing glomerulonephritis. Therefore, we treated the patient with prednisolone and cyclophosphamide having diagnosed Wegener's granulomatosis.

Bullous Necrotizing Vasculitis of the Skin

Three cases of necrotizing vasculitis clinically showing bullous skin lesions and histopathologically confirmed as leukocytoclastic vasculitis are reported. Compared with those of skin -limited non-bullous forms of cutaneous vasculitis, these cases showed relatively frequent abnormalities in urinalysis and required more aggresive corticosteriod therapy. Clinicians should be aware of the possible systemic involvements when the skin lesions are bullous in cutaneous necrotizing vasculitis.