UNASSIGNED: In suspected cases of systemic vasculitis, imaging studies should include the pulmonary artery. This is a rare case of Takayasu arteritis with a large pulmonary aneurysm. Medical management is the first line and vascular intervention if fails prior.
UNASSIGNED: Takayasu arteritis (TA) should be suspected in young women presented with hypertension, carotidynia, and claudications. Pulmonary artery involvement is frequent, occurring in 20%-50% of patients with TA. However, this case highlights the rare presentation of TA with a large pulmonary aneurysm and minimal aortic involvement. Medical management including immunosuppressive agents and biological therapies remains an important role, with vascular intervention remains as an option if medical therapy failed.

Granulomatosis with polyangiitis (GPA) is a necrotizing systemic vasculitis of small and medium-sized vessels with renal and sinopulmonary involvement. Its symptoms include chronic sinusitis, recurrent pneumonia, glomerulonephritis, constitutional symptoms, and skin manifestations with a typical onset in the fourth to sixth decade of life. We present a rare case of GPA in a 16-year-old female who presented with facial numbness and nasal regurgitation via a palatal defect. The patient reported a several-month history of recurrent epistaxis and chronic nasal congestion accompanied by several weeks of night sweats, lower right-sided facial numbness and pain, nasal regurgitation of food and liquids, and a 30-pound weight loss. A physical exam found a 3-cm defect on the right side of her palate. CT of the sinuses showed significant sinonasal destruction and petrous apicitis. GPA was confirmed via pathognomonic chest X-ray findings and biopsy results. The patient was treated with maxillary antrostomy and anterior ethmoidectomy and a follow-up was scheduled to address sequelae of the destructive sinopulmonary lesions. This case report highlights a unique presentation of GPA with an insidious development of autoimmune sinonasal destruction in an adolescent female. This presentation is rare and highlights the importance of considering autoimmune disease in cases of tissue destruction where the etiology is not apparent, even in patients at low risk for autoimmune conditions.

Immune checkpoint inhibitors (ICIs) have provided significant benefits in cancer treatment, but they could develop immune-related adverse events (irAE). ICI-associated renal adverse effects are rare and tubulointerstitial nephritis (TIN) is the most common in the renal irAE. However, only a few case reports of renal vasculitis associated with ICI have been reported. In addition, the characteristics of infiltrating inflammatory cells of ICI-associated TIN and renal vasculitis have been uncertain.
A 65-year-old man received immune checkpoint inhibitors (ICIs), anti-CTLA-4 (cytotoxic T-lymphocyte-associated protein 4) and anti-PD-1 (programmed cell death 1) antibodies for aggravated metastatic malignant melanoma. About 1 week after the second administration of nivolumab and ipilimumab, acute kidney injury developed. A renal biopsy was performed that showed TIN and non-necrotizing granulomatous vasculitis in interlobular arteries. Massive CD3+ T cells and CD163+ macrophages infiltrated both tubulointerstitium and interlobular arteries. Many infiltrating cells tested positive for Ki-67 and PD-1 ligand (PD-L1), but negative for PD-1. In CD3+ T cells, CD8+ T cells were predominantly infiltrated, and these cells were positive for Granzyme B (GrB) and cytotoxic granule TIA-1, but negative for CD25, indicating antigen-independent activated CD8+ T cells. Infiltration of CD4+ T cells was noted without obvious CD4+ CD25+ regulatory T (Treg) cells. His renal dysfunction recovered within 2 months of treatment with prednisolone in addition to discontinuation of nivolumab and ipilimumab.
We herein reported a case of ICI-related TIN and renal granulomatous vasculitis with infiltration of massive antigen-independent activated CD8+ T cells and CD163+ macrophages, and none or few CD4+ CD25+ Treg cells. These infiltrating cells might be a characteristic of the development of renal irAE.

Takayasu Arteritis (TA) is a rare form of chronic granulomatous large vessel vasculitis that is more common in Asia compared to other parts of the world. There have been several developments in the field of Takayasu arteritis in relation to genetics, classification, clinical features, imaging, disease activity assessment and management and much of these works have been done in the Asia Pacific region. We will be discussing selected few in the current review.

暂无摘要。

Takayasu\'s arteritis is a chronic granulomatous large-vessel vasculitis condition that affects the large and medium-sized arteries, primarily the heart and its major vessels. The first symptoms and indicators of Takayasu arteritis differ because the afflicted arteries are heterogeneous. Furthermore, vascular lesions might be difficult to identify at first, further complicating diagnosis. Takayasu arteritis presenting as epileptic seizures is rare. Here, we discuss a 20-year-old female who presented with a brief period of unresponsiveness, followed by a tonic stiffening, limb jerks, a postictal period of fatigue, and temporal memory loss. During the acute phase of Takayasu arteritis, high-dose glucocorticoid therapy and immunosuppressive therapy were used to control inflammatory reactions. Her symptoms gradually improved, and she was discharged from the hospital after serial monitoring; her follow-up visits revealed no recurrence.

Pathological findings are important in the diagnosis of vasculitis. However, due to the rarity of the disease, standard textbooks usually devote only a few pages to this topic, and this makes it difficult for clinicians not specializing in vasculitis to fully understand the pathological findings in vasculitis. To address the paucity of information, we present representative pathological findings in vasculitis classified in the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012). The CHCC2012 classifies 26 vasculitides into seven categories: (1) large-vessel vasculitis, (2) medium-vessel vasculitis, (3) small-vessel vasculitis, including antineutrophil cytoplasmic antibody-associated vasculitis and immune complex small-vessel vasculitis, (4) variable-vessel vasculitis, (5) single-organ vasculitis, (6) vasculitis associated with systemic disease, and (7) vasculitis associated with probable aetiology. Moreover, representative pathological findings of vasculitis-related diseases and non-inflammatory vasculopathy not mentioned in the CHCC2012 are also presented. This will be useful for clinicians to refer to typical pathological findings of vasculitis in daily practice.

OBJECTIVE: Sjögren\'s syndrome (SS) and ANCA-associated vasculitis (AAV) have distinct clinical presentation and evolution, with paucity of reports on overlap syndrome. We aimed to better characterize this entity.
METHODS: We report four additional cases from the Montpellier university hospital. We also performed a systematic literature review, according to PRISMA guidelines, in Medline, Embase, Web of science, Cochrane Library, and grey literature. Demographic, clinical, and paraclinical data on SS and AAV were analysed.
RESULTS: A total of 3133 articles was identified in databases, with 2695 articles screened for eligibility. After exclusion, we had 30 articles on 40 patients to analyse, in addition to 4 patients from our local recruitment (44 patients overall). Patients were female in 81.8%, with median age at AAV onset of 63.5 years. All patients but one presented with SS before, or concomitantly to the diagnosis of AAV, with a median delay of 12 months between both diagnoses. AAV predominantly had renal involvement (35/44 patients, 79.5%), anti-MPO antibodies being the most frequent (35 patients), even in patients presenting with granulomatosis with polyangiitis. We observed significantly more Raynaud phenomenon and associated auto-immune diseases in the group of non-granulomatous AAV (10 patients versus 1, p = 0.015 and 8 patients versus 0, p = 0.013, respectively).
CONCLUSIONS: This is the largest descriptive study on the association between SS and AAV, providing information on this challenging diagnosis and interplay between these two diseases. Particular attention should be paid in the first months after diagnosis, given the specific complications and outcomes of each disease.

暂无摘要。

Takayasu\'s arteritis (TAK) is a rare large vessel vasculitis of unknown etiology that chiefly targets the aorta and its branches. It predominantly affects females under 50 years of age. A relationship between TAK and Mycobacterium tuberculosis (TB) has been suggested for a long time, but only a few systematic studies have been done centering on this association. The present systematic review aimed to analyze the possible association between TAK and TB based on the studies conducted previously. A detailed search was conducted until April 2021 using three databases: PubMed, Cochrane Library, and MedlinePlus. PubMed search on the related topic identified 1053 articles, four on Cochrane Library, and three on MedlinePlus. Finally, 13 papers were pertinent for our review. The appropriate data was extracted from these articles, and the risk of bias assessment was done. The systematic review of these finalized articles found that the majority of the current studies supported the presence of TB in patients with TAK. Out of 13 final observational studies, only one study failed to detect a link between TAK and TB. However, data are still lacking that show a direct link between them. Future large-scale studies are needed to probe the exact role of Mycobacterium tuberculosis infection in the etiopathogenesis of TAK.