Bacterial Infections ; complications ; Diagnosis, Differential ; Factitious Disorders ; complications ; Fever of Unknown Origin ; diagnosis ; etiology ; psychology ; Humans ; Vasculitis ; complications ; Virus Diseases ; complications

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

Cerebrovascular Disorders ; diagnosis ; Diagnosis, Differential ; Humans ; Lupus Erythematosus, Systemic ; complications ; Meningitis, Bacterial ; diagnosis ; Vasculitis ; diagnosis

Behcet's disease is recognized as a multisystemic disease with various organ involvement including skin, mucous membrane, eyes, joints, veins, arteries, gastrointestinal tract, meninges, and brain. In this review, Behcet's disease has been approached from two rheumatologic perspectives--as an intermittent and periodic arthritic syndrome and as a vasculitic syndrome. Association with seronegative spondyloarthropathy and other autoimmune diseases will also be discussed.
Arthritis/etiology* ; Autoimmune Diseases/etiology* ; Behcet's Syndrome/complications* ; Human ; Vasculitis/etiology*

Arthritis, Rheumatoid/*complications ; Heart Diseases/etiology ; Lung Diseases/etiology ; Vasculitis/etiology

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications* ; Antibodies, Antineutrophil Cytoplasmic ; Eye ; Humans ; Parotid Gland

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

OBJECTIVES: To investigate the difference in the therapeutic effect of mycophenolate mofetil (MMF) or cyclophosphamide (CTX) in children with Henoch-Schönlein purpura nephritis (HSPN) of different age groups. METHODS: A retrospective analysis was conducted on the clinical data of 135 children with HSPN who were treated with MMF or CTX in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from October 2018 to October 2020. According to the immunosuppressant used, they were divided into two groups: MMF group and CTX group, and according to the age, each group was further divided into two subgroups: ≤12 years and >12 years, producing four groups, i.e, the ≤12 years MMF subgroup (n=30), the >12 years MMF subgroup (n=15), the ≤12 years CTX subgroup (n=71), and the >12 years CTX subgroup (n=19). All children were followed up for at least 12 months, and the above groups were compared in terms of clinical outcomes and the incidence rate of adverse reactions. RESULTS: There was no significant difference in the complete response rate between the MMF group and the CTX group after 3, 6, and 12 months of treatment (P>0.05). There were no significant difference in the complete response rate and the incidence rate of adverse reactions between the >12 years MMF subgroup and the ≤12 years MMF subgroup at 3, 6, and 12 months of treatment (P>0.05). The >12 years CTX subgroup had a significantly lower complete response rate than the ≤12 years CTX subgroup at 6 and 12 months of treatment (P<0.05). The >12 years CTX subgroup had a significantly higher incidence rate of adverse reactions than the >12 years MMF subgroup (P<0.05). CONCLUSIONS The efficacy and adverse reactions of MMF are not associated with age, but the efficacy of CTX is affected by age, with a higher incidence rate of adverse reactions. CTX should be selected with caution for children with HSPN aged >12 years.
Child ; Humans ; Mycophenolic Acid/adverse effects* ; IgA Vasculitis/drug therapy* ; Retrospective Studies ; Cyclophosphamide/adverse effects* ; Immunosuppressive Agents/adverse effects* ; Vasculitis/drug therapy* ; Nephritis/complications*

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications* ; Antibodies, Antineutrophil Cytoplasmic ; Humans ; Hypertrophy ; Meningitis/complications* ; Otitis Media/complications* ; Otitis Media with Effusion/complications*

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