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PubMed1989

[Vasculitis of the central nervous system].

Vasculitis of the central nervous system (CNS) is a rare disease characterized inflammation and/or sclerosis of the vascular wall, usually due to an immunological mechanism. The positive diagnosis of CNS vasculitis is difficult owing to its protean clinical manifestations, to its variable course and to the nonspecificity of CT and MRI images. Angiography itself may be of no avail since the segmental narrowing images, although suggestive, are neither constant nor pathognomonic. The causes of CNS vasculitis are very numerous. The aetiological diagnosis is easy when the causal systemic disease is already known or when an inflammatory syndrome and other localizations are present, but it is difficult in those exceptional cases where the vasculitis is limited to the CNS. Biopsy of the cortex and leptomeninges may them be required. Treatment varies according to the cause; it rests essentially on corticosteroid therapy, sometimes combined with cyclophosphamide.

Brain↗
PubMed2004

Posterior scleritis in Cogan's syndrome.

PURPOSE: To describe an unusual case of atypical Cogan's syndrome with features of posterior scleritis. METHODS: Interventional case report. RESULTS: A 53-year-old man presented with bilateral posterior scleritis, along with sensorineural hearing loss and systemic vasculitis. Systemic corticosteroid therapy was started with resolution of ophthalmic findings and improvement of vestibuloauditory symptoms. CONCLUSIONS: In the setting of atypical Cogan's syndrome, ophthalmic manifestations may be the first sign of disease.

Administration, Oral↗
PubMed1998

[Diffuse panbronchiolitis with myeloperoxidase-specific antineutrophil cytoplasmic antibody-related vasculitis].

A 46-year-old woman was referred to our department in July 1996 with complaints of fever and myalgia in her calves. She had a 20-year history of purulent sputum; diffuse panbronchiolitis had been diagnosed in 1983. Physical examination revealed low-pithed rhonchi over the lung fieldis and hypesthesia of the right leg. She had a white blood cell count of 16,100/mm3, including 4% eosinophils, and a platelet count of 80.0 x 10(4)/mm3. The serum IgE level was 2,200 U/ml, and the cold hemagglutinin titer was high. Pulmonary-function tests showed mixed ventilatory dysfunction, and arterial blood gas analysis revealed a PaO2 of 55.8 Torr on room air. Pseudomonas aeruginosa was cultured from her sputum. A chest X-ray film and CT scan showed diffuse nodular shadows and bronchiectatic changes with mild hyperinflation. An infiltrative lesion in right S6 area could also be seen. Administration of broad-spectrum antibiotics did not alleviate her symptoms. The level of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) in serum was 245 EU/ml, and 67Ga scintigraphy showed marked accumulation in the abdomen. Abdominal angiography demonstrated a bead-like appearance and irregularities in the peripheral branches of the hapatic artery, the splenic artery, the cystic artery, and the superior mesenteric artery. Because of the high MPO-ANCA level and the angiographic abnormalities, MPO-ANCA-related vasculitis was diagnosed. She was treated with 1 g of methylprednisolone daily for 3 days, followed by 60 mg of prednisolone and 50 mg of cyclophosphamide daily. Her condition improved dramatically, and the MPO-ANCA level became almost normal. During treatment, her blood pressure rose markedly with a normal serum creatinine level and normal urinalysis. Plasma renin activity was 13.3 ng/ml/hr. Renal angiography showed stenoses and irregularities in the peripheral branches of renal arteries bilaterally. These findings led to a diagnosis of renovascular hypertension due to vasculitis. Her blood pressure was controlled with an angiotensin-converting enzyme inhibitor and a calcium antagonist. Vasculitis associated with chronic supportive lung disease has occasionally been reported, which suggests a casual relation between chronic respiratory infection and ANCA-related vasculitis. Systemic vasculitis should be taken into account as a potential complication of chronic suppurative lung disease.

Anti-Inflammatory Agents↗
PubMed1989

Pulmonary capillaritis and hemorrhage. A clue to the diagnosis of systemic necrotizing vasculitis.

Patients with systemic necrotizing vasculitis frequently present as diagnostic dilemmas. In previous series of patients with polyarteritis nodosa, less than one third were diagnosed antemortem. Although current clinical awareness of systemic necrotizing vasculitis is greater than in the past and procedures for the diagnosis of these diseases have improved, patients commonly present with atypical features. The diagnosis of a systemic necrotizing vasculitis frequently remains unsuspected or unproven until an involved tissue is biopsied. We report an unusual case of systemic necrotizing vasculitis in which the diagnosis was confirmed by a transbronchial biopsy of the lung which demonstrated pulmonary capillaritis with hemorrhage.

Aged↗
PubMed2000

[Isolated vasculitis of the central nervous system].

Vasculitis is a rare cause for disease of the CNS. The isolated vasculitis of the CNS is restricted to the CNS whereas other forms of vasculitis affect various organs including the CNS. Headache, encephalopathy, focal deficits and epileptic seizures are the major symptoms suggestive for vasculitis. One major criterion of the isolated vasculitis of the CNS is the lack of evidence for other vasculitis forms or for pathology of other organs. Angiography displays multifocal segmental stenosis of intracranial vessels. MRI demonstrates multiple lesions which in part show enhancement after gadolinium. A definite diagnosis can only be made on the grounds of biopsy from leptomeninges and parenchyma. Therapy consists of corticosteroids and cyclophosphamid.

Biopsy↗
PubMed2001

Ocular manifestations of vasculitis.

Systemic necrotizing vasculitis is characterized by inflammation of blood vessels and often affects the eyes. Ocular manifestations of vasculitis may involve any part of the eye or orbit. The frequency of ocular involvement generally is dependent on the size and type of blood vessels affected by the vasculitis. This article reviews the major types of ocular inflammation and then addresses the ocular manifestations of specific systemic vasculitides.

Eye Diseases↗
PubMed1997

Vasculitis by organ systems.

Systemic vasculitides, hitherto thought to be a rare clinical entity, are now rarely considered to be an uncommon disorder and patients are often seen between several departments, suffering from a non-infectious systemic disease with multi-organ involvement. Systemic vasculitis not only poses a major management problem but also has a significant impact on healthcare resources. The clinical outcome of a vasculitic illness depends on a number of factors, such as aetiology of the vasculitic process, site, size and number of blood vessels affected, duration and severity of the disease and also the complications associated with the disease or its therapy.

Cardiovascular Diseases↗
PubMed1998

Vasculitis in systemic lupus erythematosus.

Vasculitis frequently complicates SLE, is associated with significant morbidity and mortality, and its pathophysiology is poorly understood. In seriously ill lupus patients with vasculitis, immunosuppression should almost always be considered.

Autoantibodies↗
PubMed1998

Cutaneous and systemic necrotizing vasculitis in swine.

A systemic vasculitis involving particularly the skin and kidneys has been recently described in swine under the name dermatitis/nephropathy syndrome. Twelve pigs with gross cutaneous lesions typical of this condition were necropsied, and morphologic, immunohistochemical, microbiologic, and epidemiologic characteristics were studied. The pigs were divided into three groups comprising eight pigs with acute lesions, two with chronic lesions, and two with acute lesions kept for sequential skin biopsies. Acute skin lesions consisted of round to irregular, red to purple macules and papules that often coalesced to form large, irregular patches and plaques. With time, the lesions became covered by crusts and faded gradually, sometimes leaving scars. Characteristic distribution included the perineal area of the hindquarters, limbs, dependent parts of the abdomen and thorax, and margins of the ears. In the acute phase of the disease, necrotizing and leucocytoclastic vasculitis of small-caliber blood vessels were observed within the dermis and panniculus and in various extracutaneous locations such as the renal pelvis and synovial membranes. All pigs had macroscopic evidence of pneumonia and generalized lymphadenopathy. Microscopically, they had interstitial pneumonia and perivascular cuffing of mononuclear cells in various tissues including skin. The presence of immunoglobulins and complement was demonstrated by immunofluorescence in and around necrotic vessels of the skin in the early stages. Porcine reproductive and respiratory syndrome (PRRS) virus (PRRSV) antigens were detected by immunohistochemistry in macrophages located around vessels of the tissues examined (skin and kidneys) in acute and chronic cases. PRRSV RNA was demonstrated by reverse transcription-polymerase chain reaction in lung and spleen homogenates from all pigs. The PRRSV was isolated in cell culture from 11 of the pigs. These findings suggest that PRRSV infection may play a role in the pathogenesis of this systemic vascular disease of swine.

Animals↗
PubMed2003

Temporal arteritis associated with systemic necrotizing vasculitis.

OBJECTIVE: To evaluate the clinical and laboratory characteristics of patients with systemic vasculitis associated with temporal artery involvement. METHODS: From a cohort of 120 patients fulfilling American College of Rheumatology criteria for temporal arteritis, we retrospectively identified 7 patients with systemic necrotizing vasculitis associated with histological temporal arteritis. RESULTS: Among the 7 patients, 2 had classic polyarteritis nodosa, one had unclassified systemic vasculitis, one had Wegener's granulomatosis (WG), and 3 had microscopic polyangiitis. The mean age of the patients was 70.2 years, and cranial symptoms revealed the disease in all but one patient. Temporal arteritis was generally associated with extracephalic manifestations suggestive of systemic vasculitis. Antineutrophilic cytoplasmic antibodies were positive in 3 of the 4 patients with small vessel vasculitis. Pathologically, the main temporal artery was involved in all but one patient, with inflammatory infiltrate of vasa vasorum and adventitia associated in 5 with small tributary involvement. Fibrinoid necrosis was rare, observed in 2 specimens; 2 patients with unclassified systemic vasculitis and WG had a classic giant cell arteritis (GCA) histologic pattern. Only one patient had exclusive involvement of small vessels, surrounding the spared main temporal artery. Muscle biopsies showed histopathological evidence of vasculitis in 2 patients, skin biopsy in one, and vein biopsy in the other. CONCLUSION: Temporal artery involvement in systemic necrotizing vasculitis was generally associated with extracranial clinical features suggestive of systemic vasculitis. Temporal artery biopsy is a simple tool for diagnosis of vasculitis, but the histopathological findings do not always discriminate between necrotizing vasculitis and classic GCA.

Aged↗
PubMed2004

[Vasculitis and diseases of the peripheral nervous system].

Vasculitis commonly induces peripheral neuropathies. Neuropathy is isolated in one third of the cases or participates in a multisystemic disorder. The pathological mechanism is tissular ischaemia induced by inflammation and lesions of vessel walls. Pathophysiological classification of vasculitis is mainly based on the size of affected vessels but other approaches may be proposed such as immunological classification considering the presence or absence of immunological markers. The diagnosis is supported by neuropathological examination of muscle and nerve biopsies, but electrophysiological testing may contribute to establish the diagnosis and the prognosis of the disease by showing the highly suggestive pattern of multitroncular involvement and by evaluating the extent of the neuropathy and of axonal loss. In most cases, treatment with steroids and other immunosuppressive agents result in good but partial improvement.

Electrophysiology↗
PubMedDate not supplied

Vasculitis in systemic lupus erythematosis.

Vasculitis in connective tissue diseases is not an uncommon complication. Vasculitis complicates both rheumatoid arthritis and systemic lupus erythematosis (SLE) in about 4% of cases. Cutaneous lesions, representing small-vessel involvement, are most common; however, widespread, necrotizing visceral medium-and large-vessel involvement, mimicking primary vasculitic syndromes, may also occur. Connective tissue disease-associated vasculitis is separated from primary vasculitis syndromes in classification schemes. Granulomatous large-vessel disease does not occur in connective tissue diseases, suggesting a different pathogenesis. In most disorders, the etiology of vascular inflammation in not completely understood, but basic pathogenic mechanisms can often be distinguished. The role of immune complexes in the inflammatory manifestations of SLE is recognized, and other pathogenic factors such as antineutrophil cytoplasmic antibodies, common in other vasculitides, are infrequent. A diverse spectrum of clinical features, due to inflammatory involvement of arterial and venous vessels of all sizes, characterize several connective tissue diseases including Behçet's disease and SLE. The recognition of disease manifestations due to vasculitis in these disorders has important implications for treatment and may be critical to reduce morbidity and mortality.

Humans↗
PubMed1983

Cutaneous vasculitis in systemic lupus erythematosus. A poor prognostic indicator.

The type of cutaneous lesion found in the patient with systemic lupus erythematosus is often predictive of the activity and course of the systemic disease. Vasculitis often correlates with active systemic lupus erythematosus, and portends a poor prognosis. A patient with several vasculitic changes on the fingers who died of progressive renal and central nervous system deterioration is presented.

Antibodies, Antinuclear↗
PubMed1994

Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis.

The continuing morbidity of patients with vasculitis, despite the improved prognosis with aggressive therapy, underlines the need for accurate disease assessment. We have devised a clinical index of disease activity, and evaluated its use in several forms of necrotizing vasculitis. The weighted score is based on symptoms and signs in nine separate organ systems. Disease features are only scored if they are attributable to active vasculitis. The Birmingham Vasculitis Activity Score (BVAS) was compared with two other published vasculitis activity scores, with the physician's global assessment (PGA), with outcome, and with serological markers of disease activity. In a cross-sectional study of 213 consecutive patients with different forms of vasculitis, all 107 vasculitis patients who were judged completely well on clinical assessment had a BVAS score of 0. Twenty-two patients with active vasculitis prior to treatment had a median score of 7.5 (range 4-30) and 69 with active disease on treatment had a median score of 10 (1-29). Of the 12 who died, median score immediately prior to death was 20.5 (9-30). In a serial prospective study, 30 cases had documented episodes of active disease. During periods of disease activity, the median BVAS values were significantly higher than in remission (15 [range 3-32] vs. 0 [0-2], p < 0.001); the same was true for CRP values (80 [9-361] vs. 13.5 [5-68], p < 0.001). This was not true for erythrocyte sedimentation rate (ESR), haemoglobin (Hb) or von Willebrand factor (VWF).(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗
PubMed1998

Histoplasmosis mimicking vasculitis of the central nervous system.

We describe a case of disseminated histoplasmosis that was erroneously diagnosed as central nervous system vasculitis and treated with immunosuppressive therapy. Our case emphasizes the importance of excluding infections before initiating treatment for presumed central nervous system vasculitis. Clinical clues to underlying Histoplasma infections are also discussed.

Brain Diseases↗
PubMed2004

Pharmacological therapy of vasculitis: an update.

Systemic vasculitis represents a broad range of diseases characterized by the presence of blood vessel inflammation. Immunosuppressive therapy forms the foundation of treatment for almost all forms of systemic vasculitis. Newer agents such as mycophenolate mofetil, rituximab and tumour necrosis factor-alpha inhibitors are finding new indications in the therapy of conditions such as systemic lupus erythematosus, idiopathic thrombocytopenic purpura, skin vasculitis, cytoplasmic antineutrophil antibodies-positive vasculitis, sarcoidosis, ocular inflammation, nephritis, inflammatory bowel disease and Takayasu's arteritis. However, older agents such as methotrexate, cyclophosphamide and interferon-alpha are still being explored for newer, more effective and less toxic indications in conditions such as giant cell arteritis and cutaneous polyarteritis nodosa.

Antineoplastic Agents↗