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

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

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

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

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

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*

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

OBJECTIVETo investigate the characteristic manifestations of neuropsychiatric systemic lupus erythematosus (NPSLE) in clinical practice.

METHODSA total of 198 NPSLE cases from 1998 to 2003 in Peking Union Medical College Hospital, were included. Their clinical manifestations and the results of accessory examination such as serology, lumbar puncture, head magnetic resonance imaging, and electroencephalogram were analyzed.

RESULTSThe most frequent manifestations of the NPSLE included: headache, psychiatric disorder, seizure, stroke, and acute confusional state. Fifty-two patients took head magnic resonance imaging, and 34 of them showed abnormal, including stroke attack. Among 48 patients who only had a complaint of headache, the increase of intracranial pressure and cerebral spinal fluid protein was significantly higher than other NPSLE syndromes (P < 0.05).

CONCLUSIONDiagnosis of NPSLE was mainly based on its clinical manifestations. Head magnetic resonance imaging and cerebral spinal fluid examination may be helpful. More specific accessory examinations require further development.

Adult ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Lupus Vasculitis, Central Nervous System ; cerebrospinal fluid ; diagnosis ; Magnetic Resonance Imaging ; Male