Pathogénie des vascularites associées aux ANCA en 2021 : mise au point

Anticytoplasmic neutrophil antibodies (ANCA)-associated vasculitis (AAV) are rare systemic immune-mediated diseases characterized by small vessel necrotizing vasculitis and/or respiratory tract inflammation. Over the last 2 decades, anti-MPO vasculitis mouse model has enlightened the role of ANCA, neutrophils, complement activation, T helper cells (Th1, Th17) and microbial agents. In humans, CD4T cells have been extensively studied, while the dramatic efficacy of rituximab demonstrated the key role of B cells. Many areas of uncertainty remain, such as the driving force of GPA extra-vascular granulomatous inflammation and the relapse risk of anti-PR3 AAV pathogenesis. Animal models eventually led to identify complement activation as a promising therapeutic target. New investigation tools, which permit in depth immune profiling of human blood and tissues, may open a new era for the studying of AAV pathogenesis.     

Propylthiouracil-induced autoimmune disease

Hyperthyroidism is a condition characterized by excessive production of thyroid hormones. Propylthiouracil (PTU) is commonly used as first line drug in the management of hyperthyroidism. This is a case report of 24-year-old female, a known case of hyperthyroidism since 4 years, who came with a history of fever and myalgia since 3 days and dyspnea with coughing out of blood since 1 day. Patient was taking PTU (100 mg per day) since 4 years for hyperthyroidism. Patient was immediately intubated for type-II respiratory failure. Diagnosed to be having PTU-induced autoimmune disease. PTU was stopped and treated with methylprednisolone and cyclophosphamide. Clinical features improved over a period of 8 days and discharged home successfully. Having a high suspicion for the onset of autoimmune disease in hyperthyroidism patients who are on PTU therapy and timely treatment with immunosuppressants and supportive care along with the withdrawal of the drug can make a difference in morbidity and mortality.     

Comparative analysis of Behcet's disease in the APLAR region

Aims: Behcet's disease (BD) was originally a disease of the Silk Road. Some authors think that BD from the Silk Road is different from those seen in other countries. The aim of this study was to analyze the clinical manifestations of BD in APLAR countries, where some of them are in the Silk Road (SR) and some others not (NSR). Methods: Data from Australia (NSR), Hong Kong (NSR), India (SR), Iran (SR), and Singapore (SR) were selected and analyzed under the same protocol. Prior published data from China (SR) Japan (SR) and Korea (SR) were included in the analysis. Results: The mean age at the onset of the disease was under 30 for all countries except Japan and Singapore. The male gender was more frequent except in Australia and Korea. Oral aphthosis was the most frequent manifestation (90–100%). Genital aphthosis was less frequent (57–82%). Skin manifestations were also frequent (61–87%). Ocular manifestations were reported from 21–69% of patients. The difference was mainly due to patients’ selection bias and the low number of patients in some reports. The same was true for joint (30–87%), gastrointestinal (6–38%), neurological (2.5–29%) and vascular manifestations (5–28%). Conclusion: Despite the percentage difference among some countries, the general pattern of the disease was the same, suggesting that the minor differences seen in different parts of the world were not enough to call the disease a syndrome, or to differentiate Behcet's disease of the Silk Road from those seen in Western countries.     

Possible Cerebral Vasculitis in a Case with Rheumatoid Arthritis

Cerebral rheumatoid vasculitis (CRV) is a rare, fatal, and diagnostically challenging disorder. We herein report an 81-year-old woman with a 4-year history of rheumatoid arthritis who presented with a fever, progressive disturbance of consciousness, high level of rheumatoid factor, and hypocomplementemia. The enhancement of the perforating branches in the left middle cerebral artery led us to suspect CRV. A brain biopsy could not be performed. After we intensified steroid therapy, the size of the cerebral lesions temporarily decreased. However, recurrence in the left frontal lobe occurred one month later, and the patient subsequently died. Early intensive treatments may be needed for CRV.     

Imaging Risk in Multisystem Inflammatory Diseases

Rheumatic diseases are immune-mediated inflammatory multisystem diseases with frequent cardiovascular manifestations including perimyocarditis, valvular disease, coronary artery disease, heart failure with or without preserved ejection fraction, pulmonary hypertension, aneurysms, and thrombosis. Echocardiography, carotid ultrasonography, cardiac computed tomography, cardiac magnetic resonance imaging, and positron emission tomography are valid diagnostic tools for the detection of the cardiovascular complications of the multisystem diseases that frequently determine prognosis. Furthermore, the findings of these methods may offer additive risk stratification in asymptomatic patients over the conventional risk scores used to assess cardiovascular risk in the primary prevention setting. Finally, the imaging methods offer a unique opportunity to monitor the effects of treatment on atherosclerotic lesions, coronary microcirculatory dysfunction, myocardial inflammation and fibrosis. However, studies are needed to investigate whether improvement of imaging markers by treatment or selection of treatment according to its effects on surrogate imaging markers is linked to improved prognosis.     

Decreased pulmonary perfusion in hypersensitivity pneumonitis caused by Shiitake mushroom spores

Hypersensitivity pneumonitis is an occupational hazard of mushroom workers. We describe a patient with severe hypersensitivity pneumonitis caused by spores of the Shiitake mushroom ( Lentinus edodes ) who showed a marked decrease in pulmonary perfusion, as demonstrated by pulmonary scintigraphy. This patient was treated successfully with prednisolone. These results suggest that pulmonary vasculitis may be associated with patients with hypersensitivity pneumonitis, and that steroid therapy may be clinically useful in treatment.     

改良的简易精神状态检查量表在狼疮认知筛查中的应用

目的建立系统性红斑狼疮(SLE)患者认知功能检查的床边筛查量表。方法应用改良的简易精神状态检查量表(modified mini mental status examination,MMMSE,华山医院神经内科修订版)对SLE患者进行认知功能检查,并与传统的简易精神状态检查量表(mini mental status examinmion,MMSE)检查作比较。结果①MMSE检查结果显示,神经精神性狼疮(NPSLE)组得分低于正常对照组(P＜0．01),但与SLE对照组比较差异无统计学意义(P＞0．05)；完成时间长于SLE对照组和正常对照组(P＜0．01)；SLE对照组较正常对照组在得分和完成时间上差异无统计学意义(P＞0．05)：②MMMSE检查结果显示：NPSLE组得分低于SLE对照组和正常对照组(P＜0．01),完成时间长于SLE对照组和正常对照组(P＜0．01)；而SLE对照组得分较正常对照组低,完成时间长于正常对照组,差异有统计学意义(P＜0．01)。结论MMSE一直是国内外最普及、最常用的痴呆筛查量表,但其反映认知功能损伤不够敏感,而3MSE在增加的几个项目测试中能较敏感地发现认知损伤,而且易操作、费时少(10min以内),可以用于SLE床边认知筛查。     

Fibrinogen to albumin ratio reflects the activity of antineutrophil cytoplasmic antibody‐associated vasculitis

BackgroundWe investigated whether fibrinogen to albumin ratio (FAR) at diagnosis could reflect the cross‐sectional activity and predict poor outcomes in patients with antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis (AAV).MethodsThis cross‐sectional study included 54 immunosuppressant drug‐naïve patients with AAV who had the results of plasma fibrinogen and serum albumin at diagnosis. Clinical and laboratory data at diagnosis were collected, and all‐cause mortality, cerebrovascular accident, cardiovascular disease, end‐stage renal disease occurrences were assessed as poor outcomes. FAR was calculated by the following equation: FAR = plasma fibrinogen (g/dl)/serum albumin (g/dl).ResultsThe median age was 65.5 years, and 59.3% of patients were men (33 MPA, 13 GPA and 8 EGPA). FAR was significantly correlated with Birmingham vasculitis activity score (BVAS; r = 0.271), erythrocyte sedimentation rate (ESR; r = 0.668) and C‐reactive protein (CRP; r = 0.638). High BVAS was defined as BVAS ≥16, and the cut‐off of FAR at diagnosis was set as 0.118. AAV patients with FAR at diagnosis ≥0.118 had a significantly higher risk for the cross‐sectional high BVAS than those without (RR 3.361). In the univariable linear regression analysis, CRP (β = 0.383) and FAR (β = 0.297) were significantly correlated with BVAS at diagnosis. However, in the multivariable analysis, none of them was correlated with the cross‐sectional BVAS. FAR at diagnosis could not predict poor outcomes during follow‐up in AAV patients.ConclusionsFibrinogen to albumin ratio at diagnosis could reflect the cross‐sectional BVAS but could not predict poor outcomes in patients with AAV.     

Eosinophilic granulomatosis with polyangiitis (Churg–Strauss): state of the art

Eosinophilic granulomatosis with polyangiitis (Churg–Strauss, EGPA) is a systemic small‐vessel vasculitis associated with asthma and eosinophilia. Histology of EGPA shows tissue eosinophilia, necrotizing vasculitis, and eosinophil‐rich granulomatous inflammation. EGPA commonly presents with upper airway tract and lung involvement, peripheral neuropathy, cardiac and skin lesions. Antineutrophil cytoplasmic antibodies (ANCA) are positive in ∼40% of the cases and more often in patients with clinical manifestations due to small‐vessel vasculitis. The pathogenesis of EGPA is multifactorial: the disease can be triggered by exposure to allergens or drugs, but a genetic background has also been recognized, particularly an association with HLA‐DRB4. Th2 responses are prominent, with up‐regulation of IL‐4, IL‐13, and IL‐5; however, Th1 and Th17 responses are not negligible. Eosinophils are activated, have a prolonged lifespan and probably cause tissue damage by releasing their granule proteins; their tissue recruitment can be regulated by chemokines such as eotaxin‐3 and CCL17. Humoral immunity is also dysregulated, as demonstrated by prominent IgG4 and IgE responses. EGPA promptly responds to glucocorticoid therapy, although combinations of glucocorticoids and immunosuppressants (e.g., cyclophosphamide, azathioprine) are eventually required in most cases. Newer therapeutic options include the anti‐IL5 antibody mepolizumab, whose efficacy has been described in small clinical trials, and the B‐cell‐depleting agent rituximab, reported in several case series.