暂无摘要。

BACKGROUND: Cutaneous polyarteritis nodosa (cPAN) is a systemic disease that is limited to the skin. cPAN usually presents with cutaneous reticular cyanotic, erythematous and palpable nodules, and cutaneous ulcers.Research has indicated that the use of hormones and immunosuppressive drugs can delay ulcer healing and associated neuropathy, and also elevate the risk of disease recurrence upon their reduction or withdrawal. Therefore, it is a necessary to find a safe and effective approach that minimize hormone side effects in ulcer treatment.
METHODS: The patient, a 48-year-old female of Han Chinese ethnicity, has suffered from recurrent erythema nodosum on both lower limbs for 8 years. The condition was aggravated by skin breakdown over the last 3 months. Despite multiple treatments, the patient\'s condition did not improve significantly, leading to the exploration of a combined approach of traditional Chinese and Western medicine. Following six months of combined traditional Chinese and Western medicine treatment, t the patient\'s newborn erythema and ulcers on both lower limbs did not reappear, and the ulcers gradually decreased in size and the erythema disappeared. The patient took the TCM regularly until April 15, 2023, when the ulcers were completely healed. Three months after the patient stopped taking TCM, the ulcers had completely healed with no recurrence, as observed during the follow-up visit on July 14th, 2023.
CONCLUSIONS: Traditional Chinese Medicine Combined with Low-Dose Hormones May Effectively Treat Bilateral Lower Extremity Skin Ulcers Caused by Cutaneous Polyarteritis Nodosa.

暂无摘要。

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are closely associated with neutrophil recruitment and activation, but the impact of the neutrophil apoptosis process in autoimmune disease has been rarely explained. Here, by integrating and analyzing single-cell transcriptome datasets, we found that the caspase-8-associated pathway in neutrophils was highly activated in the kidney rather than in the blood. To verify the function of caspase-8 in neutrophils on AAVs progression, we constructed neutrophil-specific caspase-8 knockout mice combined with an AAVs model induced by human ANCA from AAVs patients, a rapid and powerful model developed in this study. Our results show that caspase-8 activation of neutrophils up-regulates the expression of several inflammatory and immunoregulatory factors, especially IL23A, regulating the activation and differentiation of tissue-resident CD4+ effector memory T cells. This study reveals that the activation of caspase-8 in neutrophils can worsen glomerulonephritis of AAVs by regulating inflammation and immunity.

UNASSIGNED: To investigate the clinical characteristics of patients with systemic vasculitis in China by analyzing the data from a nationwide registry database, the Chinese Registry for Systemic Vasculitis (CRSV).
UNASSIGNED: The demographic data, clinical presentations, image and laboratory test results, disease activity assessment, treatment, and outcome of patients enrolled with the confirmed diagnosis of systemic vasculitis, including Takayasu\'s arteritis (TAK), ANCA-associated vasculitis (AAV), Behcet\'s syndrome (BD), Polyangitis nodosa (PAN), and unclassified systemic vasculitis (USV), were collected since July 2013 in the CRSV. The data during July 2013 and February 28 of 2020 were retrieved and analyzed.
UNASSIGNED: Up to February 2020, 3852 patients in total were registered in the CRSV. In Peking Union Medical College Hospital (PUMCH), 1348 patients were registered, including 730 patients with TAK, 343 with Behcet\'s disease (BD), 191 with AAV, 53 with USV, and 31 with PAN. More male patients were found in PAN (64.5%), BD (59.8%), and AAV (52.4%), while TAK was more prevalent in female patients (85.9%). The average age at disease diagnosis was 29.6 in TAK, 33.2 in BD, 28.6 in PAN, 39.4 in USV, and 48.1 in AAV. A significant difference was found between age at diagnosis of TAK and AAV. Gender comparisons revealed that the age at diagnosis of male patients with AAV was older than female patients. Hypertension (57.3%) and stroke (17.5%) were found to be more common in male patients with TAK, vascular involvement (27.3%) was found to be more common in male patients with BD, while neurological involvement (29.0%) was found to be more common in male patients with AAV than in females.
UNASSIGNED: This registry is the first in China and provides rich information about the clinical characteristics of Chinese patients with systemic vasculitis. There are significant differences in clinical presentations between different categories of systemic vasculitis.

BACKGROUND: Many patients with polyarteritis nodosa (PAN) complicated by digital gangrene have poor outcomes and related research information is limited. Our aim is to identify the associated risk and prognostic factors in PAN patients with digital gangrene.
METHODS: We conducted a retrospective study of 148 PAN patients admitted to Peking Union Medical College Hospital from Octorber 2001 to December 2018. Forty-seven (31.8%) PAN patients had digital gangrene. The average age was 40.4 ± 17.9 years.
RESULTS: The presence of digital gangrene was correlated with current smoking (P = .008, odds ratio [OR] 2.99, 95% CI, 1.33-6.73), eosinophil elevation (P = .003, OR 4.21, 95% CI, 1.62-10.91) and elevated leukocytes (P = .001, OR 4.26, 95% CI, 1.86-9.78). Thirty-two (68.1%) gangrene patients received methylprednisolone pulse therapy and all of these patients were treated with cyclophosphamide. Nine patients suffered irreversible organ injury and 2 died. Survival analysis showed higher serum C-reactive protein (CRP) was associated with poor prognosis in patients with gangrene (log-rank P = 0.042 and generalized Wilcoxon P = .020).
CONCLUSIONS: PAN patients with current smoking and eosinophil elevation were more prone to digital gangrene and a high serum CRP level predicted poor outcomes. The CRP level should be efficiently controlled to ensure a good prognosis.

暂无摘要。

Adenosine deaminases (ADAs) are enzymes of purine metabolism converting adenosine to inosine. There are two types of ADAs in humans ADA1 and ADA2. While both ADA1 and ADA2 share the same substrate, they differ in expression, cellular localization, and catalytic properties. The genetic deficiency of ADA1 results in severe combined immunodeficiency (SCID), while lack in ADA2 (DADA2) results in multiple phenotypes ranging from systemic inflammation to vascular pathology. Clinical studies have shown that the levels of ADAs in biological fluids are altered in pathophysiological conditions, suggesting that ADA activity could be a convenient marker for the diagnosis of immune diseases and cancer. Here, we describe sensitive and straightforward ELISA assays to measure ADA1 and ADA2 concentrations in biological fluids. Analysis of the serum and saliva samples from the healthy controls and DADA2 patients revealed that ADA2 enzyme concentration is significantly lower in patients than in healthy controls. In contrast, the concentration of ADA2 increases in the serum of patients with large granular leukocyte leukemia (LGLL) and patients\' saliva with head and neck cancer. Thus, this simple, non-invasive method allows for distinguishing healthy controls from the affected patient. It can be implemented in screening and diagnosis of DADA2 and follow up the treatment of LGLL and several types of head and neck cancer.

Polyarteritis nodosa (PAN) is a rare vasculitis that mainly involves small and medium arteries. It often occurs at the points where the vessels bifurcate, leading to microaneurysm formation, thrombosis, aneurysm rupture and bleeding, and infarction of organs.About a third of cases are associated with hepatitis B virus (HBV) infection.All tissues and organs of the body can be affected, with skin, joints and peripheral nerves being the most common.The pathological changes were fibrinoid necrosis, inflammatory cell infiltration and luminal thrombosis in the acute stage, and fibrous hyperplasia in the chronic stage.Overall outcomes for the disease have improved in recent decades, mainly reflecting early diagnosis and more effective treatments.The main treatments for PAN are glucocorticoid and cyclophosphamide.Patients with HBV-associated PAN should receive antiviral therapy and plasma exchange.
结节性多动脉炎（PAN）是主要累及中小动脉的一种少见的血管炎，好发于血管的分叉处，导致微动脉瘤、血栓形成、动脉瘤破裂出血及器官梗死。约1/3的患者与乙型肝炎病毒（HBV）感染相关。全身各组织器官均可受累，以皮肤、关节、外周神经最常见。病理改变急性期为血管壁纤维蛋白样坏死，炎性细胞浸润和管腔内血栓形成，慢性期为血管壁纤维性增生。近几十年来，PAN的总体预后得到了改善，主要反映了早期诊断和更有效的治疗方法。PAN治疗主要是糖皮质激素和环磷酰胺，而 HBV 相关 PAN 患者应接受抗病毒治疗和血浆置换。中华医学会风湿病学分会组织有关专家，在借鉴国内外诊治经验和指南的基础上，制定了本规范，旨在规范PAN的诊断和治疗，以降低致死率及严重并发症的发生率，更好地控制病情，改善患者预后。.

With the recognition of antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis and widespread vaccination against viral hepatitis B, the prevalence of polyarteritis nodosa (PAN) varied considerably. In our study, patients diagnosed as polyarteritis nodasa (PAN)based on the 1990 American College of Rheumatology(ACR) criteria were reclassified using 2007 European Medicines Agency(EMA) algorithm modified by 2012 Chapel Hill Consensus Conference(CHCC) definitions, aiming to evaluate the new classification criteria for the diagnosis of PAN. A total of 113 PAN patients admitted to Peking Union Medical College Hospital from January 2002 to December 2018 were retrospectively analyzed, who were classified into three subtypes including 9 patients with cutaneous, 80 with classic and 24 Hepatitis B virus (HBV) associated PAN. All patients were reclassified according to 2007 EMA algorithm using CHCC 2012 definitions. As a result, 7 patients were diagnosed as microscopic polyangiitis(MPA) and 19 patients with unclassified vasculitis based on the new classification criteria. The diagnostic rate of PAN was gradually declined as the classification criteria of vasculitides was update. However, there are quite a few PAN patients in China, whom rheumatologists should pay attention to the early diagnosis and treatment.
随着对抗中性粒细胞胞质抗体（ANCA）相关性血管炎的认识及乙型病毒性肝炎疫苗的广泛接种，结节性多动脉炎（PAN）的诊断发生了很大变化。本研究结合2012年Chapel Hill共识会议（CHCC）对PAN的定义和2007年欧洲药品管理局 （EMA）对中小血管炎分类法则，对2002年1月至2018年12月在北京协和医院住院并符合1990年美国风湿病学会（ACR）PAN分类标准的113例PAN患者重新分类，以了解新分类标准对PAN诊断的影响。113例PAN患者中皮肤型PAN者9例，经典型PAN者80例，HBV相关型PAN者24例。经过2012年CHCC PAN定义和2007年EMA中小血管炎分类法则，共有26例（23%）PAN患者被重新归类为其他系统性血管炎，7例归类为显微镜下多血管炎（MPA），19例归类为未分类血管炎。随着PAN分类法则的更新，PAN的诊断率有所下降，我国仍有一部分PAN患者，需引起重视。.