A Young Patient with Microscopic Polyangiitis Requiring Hemodialysis with Complications of Repeated Episodes of Posterior Reversible Encephalopathy Syndrome Probably Due to Different Etiologies

A young woman with microscopic polyangiitis (MPA) requiring hemodialysis showed repeated posterior reversible encephalopathy syndrome (PRES) with spatiotemporal multiple lesions over a period of two months. The first PRES episode with confusion and the second PRES episode with vertigo and nausea were caused by MPA, hypertension and renal failure. These symptoms were improved by the reinforcement of MPA treatment and blood pressure management. The third PRES episode with nausea, headache, seizure and visual changes was induced by rituximab infusion and hypertension. The PRES was improved with blood pressure and convulsant management. These conditions are challenging to diagnose and treat.     

Predicting the depressive status using empirical dietary inflammatory index in patients with antineutrophil cytoplasmic antibody‐associated vasculitis

BackgroundThis study investigated whether the empirical dietary inflammatory index (eDII) score is associated with the inflammatory burden as well as the depressive status in patients with antineutrophil cytoplasmic antibody‐associated vasculitis (AAV).MethodsEighty‐four patients with AAV participated in this study. Birmingham vasculitis activity score (BVAS) and short‐form 36‐item Health Survey mental component summary (SF‐36 MCS) were considered as indices assessing the inflammatory burden and depressive status, respectively. The eDII includes 16 food components and consists of three groups: −9 to −2, the low eDII group; −1 to +1, the moderate eDII group; and +2 to +10, the high eDII group. Furthermore, the lower eDII group includes both the low and moderate eDII groups.ResultsThe median age was 64.5 years (36 men). The eDII scores inversely correlated with SF‐36 MCS (r = −0.298, p = 0.006) but not with BVAS. SF‐36 MCS significantly differ between the lower and higher eDII groups (69.7 vs. 56.7, p = 0.016), but not among the low, moderate and high eDII groups. Additionally, when patients with AAV were divided into two groups according to the upper limit of the lowest tertile of SF‐36 MCS of 55.31, patients in the higher eDII group exhibited a significantly higher risk for the lowest tertile of SF‐36 MCS than those in the lower eDII group (RR 3.000).ConclusionWe demonstrated for the first time that the eDII could predict the depressive status by estimating SF‐36 MCS without utilising K‐CESD‐R ≥ 16 in patients with AAV.     

Acquired Immunodeficiency Syndrome (AIDS)

The Acquired Immunodeficiency Syndrome (AIDS), caused by the human immunodeficiency virus (HIV), also called the human T-lymphotropic virus type III/lymphadenopathy-associated virus [HTLV-III/LAV], has affected over 23,000 people; more than half of those with the disease have died. The actual case fatality rate approaches 100%. AIDS affects all groups and classes of people, although some are at special risk. Distribution of the disease is worldwide. The illness' effects on the body are widespread; of special interest are the ophthalmologic manifestations. The eye may be infected by various viruses (cytomegalovirus, varicella-zoster virus, herpes simples virus or HIV itself), toxoplasma gondii, candida sp, cryptococcus neoformans, M. tuberculosis, or M. avium-intracellulare. Kaposi's sarcoma may affect the eye as well. Retinal vascular abnormalities (e.g., cotton-wool spots, vasculitis) are not uncommon in AIDS. The syndrome may present with neuro-ophthalmologic manifestations. No effective treatment for the illness is currently available, although several hold promise and there is hope for an AIDS vaccine. Prevention of infection through reduction of risks appears to be the only defense against AIDS at this time.     

57例皮肤变应性血管炎的临床特点及诊治

目的探讨皮肤变应性血管炎的临床特点、早期诊断及治疗。方法对2003年12月~2004年12月接诊的57例皮肤变应性血管炎的临床表现及并发症情况进行回顾性分析,制定合理的早期诊治方案。结果归纳出典型临床特点,结合实验室检查结果,予以正确诊断并获满意治疗效果。结论早期诊断,及时对症合理的药物治疗,可以有效改善皮肤变应性血管炎的预后。     

Development of anti-neutrophil cytoplasmic antibody-associated vasculitis in a patient with Graves’ disease independent of anti-thyroid drug therapy

Abstract

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) in patients with Graves’ disease (GD) is linked with the use of anti-thyroid drugs (ATDs). We report the co-occurrence of AAV and GD in a patient that was independent of ATD therapy. A 38-year-old white male presented with systemic symptoms, palpitations, tremors, purpuric skin lesions, and digital pain. Physical examination and biological tests confirmed GD. He quickly developed multiple digital gangrenes and testicular pain/mass. Skin and testicular biopsies showed granulomatous vasculitis of the small- and medium-sized vessels, while his serum contained anti-proteinase-3 antibody.     

Anti-endothelial cell antibodies (AECA) in patients with systemic vasculitis: our research using proteomics

Importance of the field: Anti-endothelial cell antibodies (AECA) may cause damage to endothelial cell (EC) functions and therefore may be of a pathophysiological role in systemic vasculitis. The pathophysiological role of AECA, however, is still uncertain.

Areas covered in this review: To clarify the detailed roles of AECA, various methods for identification of target proteins of AECA have been developed, such as expression libraries and proteomic approaches combining two-dimensional electrophoresis and immunoblotting.

What the reader will gain: Advances, including our research, have been made in defining the target antigens of AECA, which we summarize in this review. Furthermore, we discuss the possible significance of AECA in the pathophysiology of vascular damage and the value of AECA in systemic vasculitis.

Take home message: To identify target antigens of AECA and to establish a standardized method for measuring AECA would be helpful in the search for a possible pathophysiological role of AECA in systemic vasculitis.     

WEGENER'S GRANULOMATOSIS: CLINICAL FEATURES AND OUTCOME IN SEVENTEEN PATIENTS

Seventeen patients with Wegener's granulomatosis are reviewed. Eleven males and six females, with a mean age of 46.9± 4.5 years, were followed for 35.7 ± 9.0 months. Mean duration from time of onset of symptoms to diagnosis was 8.5 ±3.1 months. Constitutional symptoms (100%), lower respiratory tract involvement (93%), renal involvement (87%), and upper respiratory tract involvement (80%) were the most frequent clinical manifestations. Arthritis (60%), dermal vasculitis (60%), and inflammatory ocular disease (40%) were also common. Elevated ESR (94%), anemia (70%), and lymphopenia (77%) were frequent laboratory findings prior to treatment. Five patients had renal failure at presentation and two patients progressed from no renal involvement at presentation to renal failure at diagnosis, while five patients progressed from renal involvement without impairment at diagnosis to end-stage renal failure. Seven patients died; six of these deaths were related to active Wegener's granulomatosis. The patients with a severe systemic vasculitis, and renal involvement had a poor outcome while predominant respiratory disease had a good prognosis.     

Renal sarcoidosis presenting as acute kidney injury with granulomatous interstitial nephritis and vasculitis

Among the various renal manifestations of sarcoidosis, granulomatous inflammation confined to the tubulointerstitial compartment is the most commonly reported finding. We present the case of a 66-year-old man with acute kidney injury, hypercalcemia, mild restrictive pulmonary disease, and neurologic signs of parietal lobe dysfunction. Kidney biopsy showed diffuse interstitial inflammation with noncaseating granulomas that exhibited the unusual feature of infiltrating the walls of small arteries with destruction of the elastic lamina, consistent with granulomatous vasculitis. The findings of granulomatous interstitial nephritis on kidney biopsy, hypercalcemia, and possible cerebral and pulmonary involvement in the absence of other infectious, drug-induced, or autoimmune causes of granulomatous disease established the diagnosis of sarcoidosis. Pulse methylprednisolone followed by maintenance prednisone therapy led to improvement in kidney function, hypercalcemia, and neurologic symptoms. Vasculocentric granulomatous interstitial nephritis with granulomatous vasculitis is a rare and under-recognized manifestation of renal sarcoidosis.     

血清抗中性粒细胞胞浆抗体滴度检测对移植肾急性血管排斥的诊断价值

动态观察了４例移植肾急性血管排斥患者经甲基泼尼松龙和环磷酰胺双冲击治疗前后血清抗中性粒细胞胞浆抗体（ＡＮＣＡ）滴度的动态变化，分析患者治疗前后临床实验室检查和肾组织病理变化。结果：（１）病情处于急性排斥期，血清ＡＮＣＡ滴度升高；（２）治疗后，临床症状减轻，肾功能改善，肾组织病理趋于正常时，ＡＮＣＡ滴度降低；（３）随访半年，移植肾排斥缓解，４例患者血清抗髓过氧化物酶抗体（ＭＰＯ－ＡＮＣＡ）均转为阴性，２例荧光法ＡＮＣＡ（ＩＦ－ＡＮＣＡ）转为阴性。结果表明，血清ＡＮＣＡ滴度的动态变化可以作为监测移植肾急性血管排斥的一项指标，对早期诊断和指导治疗有重要意义