Más difícil todavía: tratar una glomerulonefritis rápidamente progresiva grave en el seno de una neumonía por COVID-19

We present the case of a male patient with severe SARS-CoV-2 pneumonia, with simultaneous onset of p-ANCA positive rapidly progressive glomerulonephritis. We discuss the different therapeutic possibilities, emphasising the appropriateness of their administration according to the time in the course of the infection.     

Lucio phenomenon mimicking antiphospholipid syndrome: The occurrence of antiphospholipid antibodies in a leprosy patient

Lucio phenomenon is an atypical reaction of leprosy, characterized by vasculitic lesions that can mimic antiphospholipid syndrome (APS) clinically. Distinguishing the two can be difficult as antiphospholipid autoantibodies may be present in patients with leprosy. We report on a 32‐year‐old female patient presenting with a sudden onset of fever, hemorrhagic bullae, and skin necrosis on her lower legs. She was treated for APS due to the presence of antiphospholipid antibodies but had an inadequate response. A skin biopsy revealed thrombotic vasculopathy and necrotizing vasculitis associated with aggregation of foam cells in the perivascular area and subcutis, with acid‐fast bacilli in the histiocytes and blood vessel walls. Direct immunofluorescence showed IgM, C3, and fibrinogen deposition in the superficial and deep dermal blood vessels. The pathology confirmed the diagnosis of Lucio phenomenon, and appropriate therapy was given. It is essential to evaluate the patient comprehensively, including clinical, serological, and pathological aspects, to obtain the correct diagnosis.     

Primary angiitis of the central nervous system: MRI features and clinical presentation

We report the MRI features in a series of four patients with primary angiitis of the central nervous system (PACNS). Based on MRI features, clinical presentation, paraclinical investigations and laboratory tests, including cerebrospinal fluid (CSF) analysis, various differentials were considered. In two patients with MRI findings of cortical, subcortical and deep white matter lesions, lack of mass effect, focal areas of bleed and heterogeneous parenchymal, leptomeningeal or pial enhancement and a normal magnetic resonance angiography, a differential diagnosis of primary angiitis of the CNS was also considered. In all patients, an open brain biopsy was advised to establish a definitive diagnosis of PACNS. Here, we briefly discuss the MRI features, correlation with clinical presentation and paraclinical parameters for the diagnosis of this entity. We also briefly review the literature.     

Intracerebral haemorrhage as a manifestation of Lyme Neuroborreliosis?

Lyme Neuroborreliosis (LNB) has repeatedly been reported to cause cerebral vasculitis. However, there is no reliable information about the incidence of cerebral vessel affection. The majority of reports deal with ischaemic consequences, and there are a few reports of subarachnoid haemorrhage (SAH). We report a case of otherwise unexplained intracerebral haemorrhage (ICH) where clinical and laboratory tests have shown LNB. Late stage LNB might not only cause ischaemic, but haemorrhagic stroke as well.     

儿童血管炎新分类(2005年维也纳国际会议)

血管炎疾病的共同病理变化为血管壁炎症性破坏，多核、单核、嗜酸性细胞浸润，血管壁纤维蛋白样坏死。伴有肾脏病变者肾小球节段坏死并有新月体形成。     

过敏性紫癜的心肌损害

目的观察儿童过敏性紫癜并心肌损害的发生率及临床特点。方法回顾性分析93例过敏性紫癜患儿并心肌损害的临床资料，对其发生率、临床表现及其他系统损害的关系和预后进行分析。结果497例过敏性紫癜患者中有93例（18．71％）有心肌受损，主要表现为心肌酶学异常和（或）心电图异常。所有病例应用肾上腺皮质激素治疗后均治愈。结论过敏性紫癜合并心肌损害是临床较常见的表现，早期识别并及时应用激素治疗及对症处理可避免严重并发症的发生，提高治疗效果。     

Long‐standing ulcerative colitis complicated by right subclavian artery occlusion and upper limbs arteriolitis with digital ischemia

A 48‐year‐old male patient with long‐standing ulcerative colitis since February 2001 which was diagnosed by endoscopy, developed acute digital ischemia affecting both hands with fixed colour changes in the left index finger which was followed shortly by digital ulceration. Magnetic resonance angiography (MRA) of both upper limbs showed evidence of vasculitis affecting digital arterioles on both sided and right subclavian occlusion. The patient received pulse methylprednisolone followed by cyclophosphamide pulse therapy, the latter continuing on a monthly basis for 6 months with appreciable improvement and remission of the vasculitic process; follow‐up MRA showed reperfusion of the previously occluded subcalvian artery. To the authors’ knowledge vasculitis complicating the course of ulcerative colitis is a rare association and is only sporadically reported in the literature. This rare entity should be diagnosed early and aggressively treated; MRA is a very promising diagnostic tool that is suitable for both diagnosis and follow‐up of patients with this rare entity.     

Relapse rate and outcome of ANCA-associated small vessel vasculitis after transplantation.

BACKGROUND: Anti-neutrophil cytoplasm antibody-associated systemic vasculitis (AASV) is a rare disease and frequently leads to end-stage renal disease (ESRD). Potentially fatal disease activity can develop after the onset of ESRD or in transplanted patients despite the immunosuppressive effects of uraemia and rejection prophylaxis, respectively, leading to concern that such patients may have greater morbidity and mortality. METHODS: To assess the outcome of patients with AASV following kidney transplantation, a retrospective analysis was performed of nine patients with AASV at our unit who received renal transplants between 1987 and 2000. The renal survival of the patients was compared with a control population who received kidney transplants over the same period for causes other than AASV and diabetes mellitus. RESULTS: Nine patients with the diagnosis of AASV (five patients with Wegener's granulomatosis and four with microscopic polyangiitis) received eight cadaveric grafts and one live-related graft after a mean of 44 months from the start of dialysis. These patients had a mean age of 49.2 years at time of transplantation and they were followed up for a mean of 62 months post-transplantation. Two patients with Wegener's granulomatosis suffered a vasculitic relapse affecting the upper respiratory tract at 40 and 50 months post-transplantation, corresponding to a relapse rate of 0.04 per patient per year. The renal transplant function of vasculitis patients compared with 18 non-diabetic control patients who were transplanted at the same time was better in the vasculitis patients at some time points (P=0.054 at 6 months). CONCLUSIONS: There is a substantial relapse rate in the AASV population, especially affecting the upper respiratory tract and with increasing duration of follow-up. Nonetheless, renal transplantation is a good option for the treatment of vasculitis patients and their outcome compares favourably with that of other non-diabetic patients following transplantation.