The common clinical manifestations of the primary agiitis of the central nervous system include burst of headache, dementia, change of aptitude, paralysis of cranial nerves, and recurrent focal depletion of the neural function. Lptomeningeal and brain biopsy are still the gold criteria for diagnosis. The prognosis may be improved after cortin and immunosuppressant therapy.
Humans ; Vasculitis, Central Nervous System ; diagnosis ; pathology ; therapy

A rare case of polyangiitis overlap syndrome is described. The patient was a 25-year-old man who had palpable purpura on his legs which showed leukocytoclastic vasculitis, and polyarteritis nodosa. Superior mesenteric arteriography showed microaneurysms in jejunal branches with focal segmental necrotizing arteritis of small and medium sized muscular arteries in the jejunum. Deposits of IgA and C3 in the superficial blood vessels of the lesional skin were consistent with the features of Henoch-Schonlein purpura. The patient died about two months after initial admission in spite of cytotoxic agent and steroid administration.
Adult ; Case Report ; Human ; Male ; Polyarteritis Nodosa/*complications/pathology ; Vasculitis, Hypersensitivity/*complications/pathology

Acute hemorrhagic edema of infancy is an unusual form of leukocytoclastic vasculitis occuring in children from the age 4 months to 2 years. The etiology remains unknown. Numerous studies, however, suggest acute hemorrhagic edema of infancy as an immune-mediated vasculitis in response to a variety of antigenic stimuli. We report a case of an acute hemorrhagic edema of infancy; 11-month-old boy with a history of fever for 3 days and a history of purpuric rash on the extremities, trunk, buttock and oral mucosa for 2 days.
Acute Disease ; Biopsy ; Edema/immunology/*pathology ; Exanthema/immunology/pathology ; Hemorrhage/immunology/*pathology ; Human ; Infant ; Male ; Vasculitis, Hypersensitivity/immunology/*pathology

OBJECTIVETo evaluate the clinical and coronary angiographic features of patients with systemic vasculitis and coronary artery disease.

METHODFifteen patients (11 male) with systemic vasculitis and coronary artery diseases admitted to our hospital from January 1999 to October 2009 were reviewed.

RESULTSThere were 6 patients with Behcet's disease, 3 patients with Churg-Strauss syndrome, 2 patients with Takayasu arteritis, 1 patient with polyarteritis nodosa, 1 patient with microscopic polyangiitis, 1 patient with Wegner's granulomatosis and 1 patient with Kawasaki disease. Mean age of this cohort was (39.3 ± 11.9) years. Adverse coronary events occurred in 4 patients during the inactive phase of systemic vasculitis and in 9 patients during the active phase of systemic vasculitis. Twelve patients were hospitalized with acute myocardial infarction, 2 with angina pectoris and 1 with cardiac tamponade. There were 3 patients with acute left ventricular dysfunction and 3 patients with severe arrhythmias. Compared to patients in the inactive phase, patients in the active phase were younger [(32.4 ± 8.1) years vs. (47.0 ± 10.2) years], had less risk factors for atherosclerosis (1.2 ± 1.5 to 2.8 ± 1.7) and the time intervals between coronary artery disease and systemic vasculitis was shorter [0 - 7 years (average 1.6 years) to 3 - 30 years (average 17.7 years)]. Coronary angiography evidenced coronary stenosis or occlusions in 11 patients, coronary aneurysm and acute thrombosis in 1 patient, coronary aneurysms and occlusions in 1 patient and coronary spasm in 2 patients. LVEF measured by echocardiography was less than 50% in 8 patients.

CONCLUSIONPatients with various systemic vasculitis could develop severe coronary artery disease due to coronary stenosis/occlusion, aneurysma, thrombosis and coronary spasm.

Adult ; Coronary Angiography ; Coronary Artery Disease ; complications ; pathology ; Coronary Vessels ; pathology ; Female ; Humans ; Male ; Middle Aged ; Vasculitis ; complications ; pathology

OBJECTIVETo study the clinical and pathologic features of Churg-Strauss syndrome (CCS).

METHODSThree cases of Churg-Strauss syndrome, including 1 autopsy case and 2 cases with open thoracoscopic lung biopsy, were retrospectively reviewed. All the tissue samples were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin.

RESULTSThe first patient was a 68-year-old man who had history of asthma for 4 years, with recent exacerbation and chest pain for 2 weeks. Patient died 1 day after admission due to myocarditis and myocardial infarction. He did not have peripheral eosinophilia, skin or paranasal sinus pathology. CSS represented an incidental autopsy finding and he had never been treated with corticosteroid before. The other 2 patients were a 58-year-old male and a 12-year-old female, respectively. Both had history of asthma, peripheral eosinophilia and lung consolidations on computed tomographic examination. Pathologically, all cases showed vasculitis, perivascular allergic-type granulomas, eosinophilic pneumonia and asthmatic bronchitis.

CONCLUSIONSThorough understanding of the clinical and pathologic criteria is essential for arriving at a correct diagnosis of CSS. Although some patients may present with atypical symptoms, lung biopsies often reveal the classic histologic findings which include vasculitis and perivascular allergic granuloma formation.

Aged ; Churg-Strauss Syndrome ; pathology ; Eosinophilia ; pathology ; Female ; Granuloma ; pathology ; Humans ; Lung Diseases ; pathology ; Male ; Middle Aged ; Pulmonary Eosinophilia ; pathology ; Vasculitis ; pathology

Nonsystemic vasculitic neuropathy (NSVN) is a localized vasculitis confined to the peripheral nerves. Absence of systemic manifestations frequently leads to mis- or under diagnosis without the aid of pathologic study. NSVN may present typically with multiple mononeuropathies or less commonly with a sensorimotor polyneuropathy. We report two cases of NSVN presenting with acute severe painful neuropathy. Sural nerve pathology showed unequivocal vasculitis. High dose corticosteroid therapy was effective in controlling the pain. NSVN should be considered as a treatable cause of acute painful neuropathy.
Acute Pain* ; Diagnosis ; Mononeuropathies ; Pathology ; Peripheral Nerves ; Polyneuropathies ; Sural Nerve ; Vasculitis

BACKGROUND: Cutaneous manifestations of vasculitis can be seen as pleomorphic lesions; purpura, erythema, nodule, bulla, ulcer and so on. In cases of polyarteritis nodosa (PAN), cutaneous presentations of small artery pathology may be seen in about one fourth among those patients with systemic form of PAN, and in all cases of cutaneous form subset. OBJECTIVE: To examine the pattern or morphology of cutaneous lesions found in the skin (especially on the lower legs) among Korean patients with cutaneous form of PAN. Patients AND METHODS: Eight patients with diagnosis-confirmed cases of cutaneous PAN were examined regarding the patterns of cutaneous lesions, as well as possible local symptoms, distributions, duration, and any positive findings in laboratory examinations and systemic review. RESULTS: Clinical patterns of cutaneous lesions observed in the lesional areas among those 8 patients were mottled or atypical reticular erythema (5 cases), subcutaneous nodules (3 cases), scattered erythematous patch (3 cases), ecchymotic erythematous patch (3 cases) and superficial ulcer (1 case). Tenderness was detected at the nodular lesions; predilection site were shin and calf areas; a few laboratory abnormalities and systemic symptoms were found regardless of the duration of skin lesions. CONCLUSION: Common clinical patterns of cutaneous lesion recognized with 8 patients of cutaneous PAN were mottled/atypical reticular erythemas, subcutaneous nodules, erythematous/ecchymotic patches, and these in all cases were seen at the lower legs. In patients with each different clinical presentation, there were no relevancies between the duration and severity of the disease.
Arteries ; Erythema ; Humans ; Leg* ; Pathology ; Polyarteritis Nodosa* ; Purpura ; Skin ; Ulcer ; Vasculitis

Extracorporeal Membrane Oxygenation ; methods ; Female ; Humans ; Middle Aged ; Vasculitis ; diagnosis ; pathology ; therapy

Acute Disease ; Chronic Disease ; Humans ; Male ; Middle Aged ; Vasculitis, Leukocytoclastic, Cutaneous ; pathology

We report a 46-year-old woman with primary biliary cirrhosis (PBC) presenting with Sjogren's syndrome and systemic mononuclear inflammatory vasculopathy. Biopsy specimens of sural nerve showed findings consistent with vasculitic neuropathy. Perivascular inflammatory mononuclear cell infiltration was observed on muscle biopsy specimen. The findings of abdominal computed tomography and brain magnetic resonance imaging were suggestive of vasculitis. Clinical manifestations and radiologic findings were improved after high dose prednisolone therapy.
Biopsy, Needle ; Case Report ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Liver Cirrhosis, Biliary/diagnosis+ACo- ; Liver Cirrhosis, Biliary/complications+ACo- ; Middle Age ; Prednisone/administration +ACY- dosage ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/diagnosis+ACo- ; Sjogren's Syndrome/complications ; Sural Nerve/pathology ; Treatment Outcome ; Vasculitis/pathology ; Vasculitis/drug therapy ; Vasculitis/diagnosis+ACo- ; Vasculitis/complications