systemic necrotizing vasculitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis accompanied by granulomas and eosinophilic inflammation, exhibiting marked peripheral blood eosinophiliaandasthma. Neuropathy is a difficult-to-treat common manifestation that frequently remains after achieving clinical remission with current therapy in a subpopulation of patients with EGPA with or without life-threatening organ involvement. Refractory neuropathy regularly reduces the quality of life and requires glucocorticoids (GCs) and/or immunosuppressants for a long time...

In this article, we present an unusual case of granulomatosis with polyangiitis (GPA) in a 41-year-old man. The initial presentation of the disease was atypical, with persistent fever, cough, and fatigue, accompanied by elevated inflammatory markers in association with a large, solitary lung lesion observed at the chest X-ray. Despite the presence of an initial radiological picture suggesting pneumonia, the lack of response to antibiotics necessitated a more in-depth evaluation. The diagnosis was confirmed through a lung biopsy and serological tests positive for anti-neutrophil cytoplasmic antibodies (c-ANCA)...

OBJECTIVES: Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) are life-threatening systemic autoimmune diseases manifesting in the kidneys as necrotizing crescentic glomerulonephritis (NCGN). ANCA antigens are myeloperoxidase (MPO) or proteinase 3. Current treatments include steroids, cytotoxic drugs and B cell-depleting antibodies. The use of chimeric antigen receptor (CAR) T cells in autoimmune diseases is a promising new therapeutic approach. We tested the hypothesis that CAR T cells targeting CD19 deplete B cells, including MPO-ANCA-producing B cells, thereby protecting from ANCA-induced NCGN...

Primary angiitis of the central nervous system (PACNS) is a rare vasculitis in the central nervous system. Herein, we report a case of diagnosis and treatment of necrotic pattern PACNS, which was difficult to differentiate from a brain abscess. A 19-year-old male presented with blurred vision and a headache. Brain MRI revealed irregular rim-enhancing necrotic masses with central diffusion-high signal intensity in the corpus callosum and peripheral diffusion-high signal intensity in the left parietotemporal periventricular area...

OBJECTIVE: To investigate the significance of anti-histidyl tRNA synthetase (Jo-1) antibody in idiopathic inflammatory myopathies (IIM) and its diseases spectrum. METHODS: We enrolled all the patients who were tested positive for anti-Jo-1 antibody by immunoblotting in Peking University People's Hospital between 2016 and 2022. And the patients diagnosed with anti-synthetase antibody syndrome (ASS) with negative serum anti-Jo-1 antibody were enrolled as controls...

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a heterogeneous group of rare diseases characterized by necrotizing inflammation predominantly of small vessels and the presence of these circulating antibodies. AAV comprises three important diseases, namely granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis, which affect multiple organ systems, significantly affecting patients' quality of life and survival. The diagnosis is established according to the clinical manifestations, detectable ANCA, and histopathology findings...

BACKGROUND: Sarcoidosis affects multiple organs and exhibits diverse clinical manifestations. Although tubulointerstitial nephritis is a known feature of renal involvement, necrotizing vasculitis is rare. Furthermore, prostate involvement with urinary retention is unusual in patients with sarcoidosis. Here, we report a case of systemic sarcoidosis with a rare combination of manifestations and different acute kidney injuries. CASE PRESENTATION: A 66-year-old man developed sudden urinary retention and fever...

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic autoimmune disease characterized by necrotizing inflammation of small blood vessels. Glucocorticoids (GC) in combination with rituximab or cyclophosphamide can reduce AAV-related mortality and rescue renal function. However, several side effects associated with these agents, including GC toxicity, are concerning. Avacopan, an inhibitor of the C5a receptor, is now available for AAV treatment and is expected to mitigate GC toxicity...

Polyarteritis nodosa (PAN), also known as panarteritis nodosa, represents a form of necrotizing vasculitis that predominantly affects medium-sized vessels, although it is not restricted to them and can also involve smaller vessels. The clinical presentation is heterogeneous and characterized by a significant number of patients exhibiting general symptoms, including asthenia, fever, and unintended weight loss. Although PAN can involve virtually any organ, it preferentially affects the skin, nervous system, and the gastrointestinal tract...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and necrotizing vasculitis of small and medium vessels with a heterogeneous presentation, multiorgan involvement, characterized by the presence of chronic rhinosinusitis, asthma, and peripheral eosinophilia. Nervous system involvement is frequent, especially in the peripheral nervous system (PNS), in the form of mononeuritis multiplex. However, subarachnoid and cerebral hemorrhage, cerebral infarction, cranial nerve palsies, and cortical blindness have also been reported in the literature...

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis common in males over 50 years of age that causes various organ symptoms. In recent years, it has become important to distinguish deficiency of adenosine deaminase 2 (DADA2) from childhood-onset PAN. A 13-year-old girl was urgently transferred to our hospital with sudden weakness in her right upper and lower limbs. The National Institutes of Health Stroke Scale (NIHSS) was 8. Plain MRI of the brain indicated high-signal areas in the right caudate nucleus, internal capsule, and left basal ganglia when applying T2-weighted, fluid-attenuated inversion recovery (FLAIR), and diffusion-weighted imaging (DWI); and low signals in the same regions in an apparent diffusion coefficient (ADC) map...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic necrotizing vasculitis affecting small-to-medium-sized vessels. EGPA's clinical manifestations are heterogeneous, affecting different organs and systems, and the upper respiratory tract can be affected by ear, nose and throat (ENT) involvement. The aim of our study was to assess type manifestations at the time of diagnosis in a cohort of EGPA patients and correlate findings with baseline variables (sex, age, antineutrophil cytoplasmic antibodies-ANCA-status) and literature reports...

BACKGROUND: Pediatric Behçet's disease (PBD) is rarer than BD and can be a challenging diagnosis as clinical picture may be incomplete. As in adult patients, sight-threatening ocular manifestations may lead to diagnosis. In this study, we aimed to report a series of cases of PBD with ocular manifestations and provide a review of the literature. METHODS: Retrospective case series of PBD patients with ocular manifestations. Demographic, ophthalmological and systemic data at presentation and during follow-up were collected and analyzed...

Granulomatosis with polyangiitis (GPA), previously Wegener's granulomatosis, is a necrotizing vasculitic disease process affecting the small- and medium-sized blood vessels. GPA is frequently associated with damage to the respiratory tract and kidneys but often affects other organ systems including the eyes, nasopharynx, and nervous system. Due to the vague nature of presenting symptoms and the progressive nature of GPA, it is essential to keep a broad differential to mitigate the high morbidity and mortality associated with the disease...

Primary angiitis of the central nervous system (PACNS) is a rare and potentially severe form of vasculitis that is limited to the brain, spinal cord, and meninges. Despite extensive research, the etiology and underlying immunologic mechanisms of PACNS remain largely unknown. PACNS presents with a variety of clinical, radiological, and pathologic features, but it is generally characterized by inflammation and destruction of the walls of blood vessels in the CNS, which can lead to tissue ischemia and/or hemorrhage...

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis with a poor prognosis, characterized by inflammation and necrosis of medium-sized arteries. PAN patients can present with a wide range of systemic manifestations, whereas cutaneous arteritis (CA) is a restricted manifestation to skin of the disease with a more favorable prognosis. Thus, differentiation between PAN and CA is crucial. Here we present two cases that were initially diagnosed as CA due to the limited presence of systemic symptoms, but were finally diagnosed as PAN through catheter-based angiography...

INTRODUCTION: Cryoglobulinemic vasculitis is a type of small vessel vasculitis diseases that can cause dysfunction in multiple organs. It is characterized by general symptoms, often accompanied by nonspecific cutaneous, articular, neurological, and renal manifestations. Diagnosing cryoglobulinemia through biological testing can be time-consuming and sometimes yields negative results, making diagnosis challenging. There are also other potentially life-threatening complications that can significantly impact prognosis and delay urgent treatment, including digestive manifestations and heart failure...

Juvenile gangrenous vasculitis is characterized by the abrupt onset of scrotal ulcerations in young males, preceded by fever and pharyngeal symptoms. The etiology of this disease is poorly understood. The course is benign and self-limiting within a few weeks with no relapse. Because of its rare incidence, physicians often confuse it with Fournier's gangrene, which progresses rapidly to severe systemic symptoms requiring urgent surgical intervention. Herein, we report a rare case of juvenile gangrenous vasculitis of the scrotum and emphasize the importance of awareness of this diagnosis to avoid unnecessary invasive surgical intervention...

BACKGROUND: Previous case series have reported idiopathic eosinophilic vasculitis as a potential manifestation of hypereosinophilic syndrome (HES). This condition is characterized by digital necrotizing, systemic vasculitis that affects varying-sized blood vessels. This report presents our experience in treating a patient with eosinophilic vasculitis. CASE PRESENTATION: We describe the case of a 23-year-old man who presented with idiopathic HES, which manifested as digital ulcers and peripheral ischemia in both the upper and lower limbs, without the involvement of other organ systems...

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe cutaneous adverse reaction involving multiorgan failure, with a complex interaction of various drugs, human herpesvirus reactivation and immune abnormalities suggested as the aetiology. We herein present the case of a 70-year-old man with a one-week history of fever, facial oedema, erythematous macules and purpura on his trunk and extremities. He had anti-TIF1γ antibody-positive dermatomyositis and was treated with prednisolone sodium succinate (20 mg/day)...