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1.
Front Immunol ; 15: 1354349, 2024.
Article in English | MEDLINE | ID: mdl-38707895

ABSTRACT

Antiphospholipid antibodies (aPL) are both laboratory evidence and causative factors for a broad spectrum of clinical manifestations of antiphospholipid syndrome (APS), with thrombotic and obstetric events being the most prevalent. Despite the aPL-triggered vasculopathy nature of APS, vasculitic-like manifestations rarely exist in APS and mainly appear associated with other concurrent connective tissue diseases like systemic lupus erythematous. Several studies have characterized pulmonary capillaritis related to pathogenic aPL, suggesting vasculitis as a potential associated non-thrombotic manifestation. Here, we describe a 15-year-old girl who develops hepatic infarction in the presence of highly positive aPL, temporally related to prior non-severe COVID-19 infection. aPL-related hepatic vasculitis, which has not been reported before, contributes to liver ischemic necrosis. Immunosuppression therapy brings about favorable outcomes. Our case together with retrieved literature provides supportive evidence for aPL-related vasculitis, extending the spectrum of vascular changes raised by pathogenic aPL. Differentiation between thrombotic and vasculitic forms of vascular lesions is essential for appropriate therapeutic decision to include additional immunosuppression therapy. We also perform a systematic review to characterize the prevalence and clinical features of new-onset APS and APS relapses after COVID-19 for the first time, indicating the pathogenicity of aPL in a subset of COVID-19 patients.


Subject(s)
Antibodies, Antiphospholipid , Antiphospholipid Syndrome , COVID-19 , SARS-CoV-2 , Vasculitis , Humans , COVID-19/complications , COVID-19/immunology , Female , Adolescent , Antibodies, Antiphospholipid/blood , Antibodies, Antiphospholipid/immunology , Vasculitis/immunology , Vasculitis/etiology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/immunology , SARS-CoV-2/immunology , Liver/pathology
2.
Pathologica ; 116(2): 119-133, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38767544

ABSTRACT

The mechanisms underlying the onset and progression of vasculitis remain poorly understood. This condition is characterized by damage to the vascular wall, recruitment of inflammatory cells, and subsequent structural remodeling, which are hallmarks of vasculitis. The histopathological classification of vasculitis relies on the size of the affected vessel and the predominant type of inflammatory cell involved - neutrophils in acute cases, lymphocytes in chronic conditions, and histiocytes in granulomatous forms. Pathological changes progress in every context, and a single vasculitic pattern can be associated with various systemic conditions. Conversely, a single causative agent may lead to multiple distinct clinical and pathological manifestations of vasculitis. Moreover, many cases of vasculitis have no identifiable cause. A foundational understanding of the normal structure of the cutaneous vascular network is crucial. Similarly, identifying the cellular and molecular participants and their roles in forming the "dermal microvascular unit" is propedeutical.This review aims to elucidate the complex mechanisms involved in the initiation and progression of vasculitis, offering a comprehensive overview of its histopathological classification, underlying causes, and the significant role of the cutaneous vascular network and cellular dynamics. By integrating the latest insights from studies on NETosis and the implications of lymphocytic infiltration in autoimmune diseases, we seek to bridge gaps in current knowledge and highlight areas for future research. Our discussion extends to the clinical implications of vasculitis, emphasizing the importance of identifying etiological agents and understanding the diverse histopathological manifestations to improve diagnostic accuracy and treatment outcomes.


Subject(s)
Skin , Vasculitis , Humans , Vasculitis/pathology , Vasculitis/etiology , Skin/pathology , Skin/blood supply , Neutrophils/pathology , Lymphocytes/pathology , Lymphocytes/immunology , Skin Diseases, Vascular/pathology , Skin Diseases, Vascular/immunology , Skin Diseases, Vascular/etiology , Skin Diseases, Vascular/diagnosis
4.
Clin Immunol ; 263: 110207, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38608995

ABSTRACT

Behçet's disease (BD) is an autoinflammatory disease with multifactorial and polygenic etiology, potentially involving arteries and veins of any size resulting in variable vessel vasculitis. We report a case of an Iranian male who presented with porto-sinusoidal vascular disorder due to venous vasculitis as initial manifestation of BD. Despite immunosuppression, anticoagulation and venous recanalization, he subsequently developed severe nephrotic-range proteinuria mimicking a primary renal disease which was completely and immediately ameliorated by stenting of the vena cava. This demonstrates that the proteinuria was caused by increased intraglomerular pressure due to venous outflow obstruction as a consequence of venous vasculitis. To our knowledge, this is the first report of massive proteinuria caused by venous obstruction of the caval vein in the context of BD. Altogether, this case demonstrates the extensive spectrum of vascular disease in BD.


Subject(s)
Behcet Syndrome , Proteinuria , Humans , Behcet Syndrome/complications , Male , Proteinuria/etiology , Vasculitis/etiology , Adult
9.
J Dermatol ; 51(2): 150-159, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37955334

ABSTRACT

Autoinflammatory diseases (AIDs) characterized by recurrent episodes of localized or systemic inflammation are disorders of the innate immune system. Skin lesions are commonly found in AIDs and cutaneous vasculitis can coexist with AIDs and even present as the most striking feature. This review aims to focus on the frequent cutaneous vasculitis association in three monogenic AIDs including familial Mediterranean fever (FMF), deficiency of adenosine deaminase type 2 (DADA2), and the recently identified adult-onset VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome. Cutaneous vasculitis in FMF is characterized by: (1) small-vessel vasculitis similar to IgA vasculitis with palpable purpura but increased intussusception complication and less vascular IgA deposit, and (2) cutaneous arteritis-like vasculitis presenting as subcutaneous nodules most often with higher glomerular involvement. DADA2 has a wide spectrum of clinical presentations ranging from fatal systemic vasculitis with multiple strokes, especially in pediatric patients, to limited cutaneous disease in middle-aged patients. DADA2 shares similar clinical and histopathological features with polyarteritis nodosa (PAN). As a result, DADA2 is commonly initially misdiagnosed as childhood PAN. Livedo racemosa reveals the most common cutaneous manifestation of cutaneous vasculitis in patients with DADA2. VEXAS syndrome is a life-threatening disease. A diagnosis of VEXAS syndrome should be strongly considered or could be made in patients with skin lesions characterized by Sweet syndrome-like eruption, livedo racemosa, concomitant relapsing polychondritis, deep venous thrombosis, pulmonary involvement, and progressive hematologic abnormalities such as myelodysplastic syndrome with a unique finding of cytoplasmic vacuoles in myeloid and erythroid precursor cells from bone marrow aspirate smear. As skin involvement is common in AIDs and may present as the most frequent manifestation, especially in DADA2 (70% to 90%) and VEXAS syndrome (83% to 91%), dermatologists play a crucial role in contributing to the early diagnosis of these AIDs with early initiation of the appropriate therapy to avoid progressing fatal outcomes.


Subject(s)
Agammaglobulinemia , Familial Mediterranean Fever , Livedo Reticularis , Myelodysplastic Syndromes , Polyarteritis Nodosa , Severe Combined Immunodeficiency , Skin Diseases, Genetic , Skin Diseases , Vasculitis , Adult , Humans , Child , Middle Aged , Adenosine Deaminase/genetics , Livedo Reticularis/complications , Intercellular Signaling Peptides and Proteins , Vasculitis/diagnosis , Vasculitis/etiology , Polyarteritis Nodosa/complications , Polyarteritis Nodosa/diagnosis , Skin Diseases/diagnosis , Skin Diseases/etiology , Familial Mediterranean Fever/diagnosis , Mutation
10.
Curr Opin Rheumatol ; 36(1): 27-34, 2024 01 01.
Article in English | MEDLINE | ID: mdl-37916482

ABSTRACT

PURPOSE OF REVIEW: Cryoglobulinemic vasculitis (CV) is an immune complex mediated small vessel vasculitis characterized by the presence of cryoglobulins in serum, often associated with hepatitis C infection, systemic autoimmune diseases or hematological conditions. The focus of this review is to provide an update on new insights into pathogenesis, epidemiology and therapies of infectious and noninfectious type II and type III CV. RECENT FINDINGS: The introduction of new antiviral drugs for treatment of hepatitis C infection implied major changes in HCV-related CV, allowing to shed new lights on CV pathogenesis and mechanisms of relapse and, therefore, to increase the relevance of autoimmune diseases in CV epidemiology. Specific B-cell clones are involved in the production of pathogenic immune complexes that leads to small-vessel vasculitis. Therefore, both antiviral treatments [direct-acting antivirals (DAAs) and oral nucleot(s)ide analogues] and targeted anti-CD20 therapies (rituximab) prove to be safe and effective options, leading to a better prognosis. Association of Sjögren syndrome and CV defines a specific phenotype of patients, characterized by severe manifestations and poor outcome. SUMMARY: Removing viral stimulation on B-cells through direct-acting antivirals and blocking B-cells proliferation and differentiation with rituximab are the goals of treatment of CV. However, further research is needed to identify prognostic factors of refractory and relapsing disease.


Subject(s)
Cryoglobulinemia , Hepatitis C, Chronic , Hepatitis C , Vasculitis , Humans , Rituximab/therapeutic use , Antiviral Agents/therapeutic use , Hepatitis C, Chronic/complications , Hepatitis C/complications , Vasculitis/drug therapy , Vasculitis/etiology , Cryoglobulinemia/etiology , Cryoglobulinemia/complications , Hepacivirus
11.
Zh Nevrol Psikhiatr Im S S Korsakova ; 123(12. Vyp. 2): 5-11, 2023.
Article in Russian | MEDLINE | ID: mdl-38148691

ABSTRACT

The review considers the clinical picture, key aspects of the diagnosis and treatment of vasculitis that are the causes of strokes (giant cell arteritis, polyarteritis nodosa, varicella zoster virus vasculopathy, cerebrovascular pathology caused by herpes simplex virus types 1 and 2, primary CNS angiitis, adenosine deaminase-2 deficiency).


Subject(s)
Stroke , Vasculitis , Humans , Vasculitis/diagnosis , Vasculitis/etiology , Vasculitis/pathology , Stroke/complications , Stroke/diagnosis , Herpesvirus 3, Human
12.
Front Immunol ; 14: 1271584, 2023.
Article in English | MEDLINE | ID: mdl-37901234

ABSTRACT

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. Case presentation: We report the case of a 60-year-old male patient with a medical history of rheumatoid arthritis. He was admitted to the Nephrology Department for investigation of necrotic vascular purpura, acute kidney injury, and pancytopenia. Laboratory tests revealed consumption of the C3 and C4 complement fractions and the presence of mixed-type III cryoglobulinemia. Despite the initiation of the treatment, the patient rapidly developed multiple severe organ failures, including renal, cardiac, respiratory, and finally digestive complications. Acute colic ischemia led to emergency surgery and the patient was transferred to the Intensive Care Unit. Despite surgical intervention and hemodynamic support, the patient experienced multi-visceral organ failure and died two hours after admission. Discussion: Mixed cryoglobulinemia vasculitis may result in rare cases of acute and life-threatening organ damage, such as cardiac or respiratory failure with pulmonary hemorrhage, gastrointestinal ischemia, and neurological disorders. These severe manifestations are associated with a poor prognosis and it is crucial to promptly initiate an aggressive therapeutic strategy.


Subject(s)
Cryoglobulinemia , Vasculitis , Male , Humans , Middle Aged , Cryoglobulinemia/etiology , Cryoglobulinemia/complications , Vasculitis/etiology , Vasculitis/complications , Complement C4 , Prognosis , Multiple Organ Failure/etiology , Ischemia/complications
13.
Med Arch ; 77(4): 310-313, 2023.
Article in English | MEDLINE | ID: mdl-37876567

ABSTRACT

Background: Multisystem Inflammatory Syndrome of children (MIS) is a pathological condition that occurs in response to a SARS-CoV-2 infection, the syndrome has been described as a "Kawasaki disease"-like illness and the spectrum of associated abnormalities, including vascular complications, remain to be fully defined. Objective: The aim of this article was to present a case of MISC presented with limping and associated with large vessel vasculitis. Case presentation: In this article we present a case of 10-year-old male presented to emergency department complaining of limping of one-week duration and left hip pain, associated with high grade prolonged fever, abdominal pain and weight loss. The patient was ill looking, couldn't bear weight and was admitted to pediatric intensive care unit. Laboratory workup have rule out infectious and malignant causes as well as known rheumatological causes. Inflammatory markers were elevated. Ultrasound, Doppler ultrasound, CT scan of the affected hip showed evidence of vasculitis extending from the left femoral artery reaching the left common iliac artery with intramural thrombus. According to WHO criteria the patient diagnoses was MIS-C. treatment was started immediately with IVIG and steroids in addition to anticoagulants, dramatic improvement was noticed within 24 hours. Patient was discharged after 10 days of hospitalization. Conclusion: MIS-C is a new emerging medical diagnosis after the pandemic of COVID-19. it is described a Kawasaki-like syndrome that affect small to medium vessels. This case reports a large vessel vasculitis associated with MIS-C, it helps the understand the extend of this new syndrome and the variety of complaints that may result from large vessels involvement.


Subject(s)
COVID-19 , Vasculitis , Child , Male , Humans , Systemic Inflammatory Response Syndrome/complications , Systemic Inflammatory Response Syndrome/diagnosis , COVID-19/complications , COVID-19/diagnosis , Vasculitis/diagnosis , Vasculitis/drug therapy , Vasculitis/etiology , Gait
14.
Reumatismo ; 75(3)2023 Sep 18.
Article in English | MEDLINE | ID: mdl-37721348

ABSTRACT

Adenosine deaminase 2 deficiency (DADA2) is a rare monogenic vasculopathy caused by loss-of-function homozygous or compound heterozygous mutations in ADA2, formerly CECR1 (cat eye syndrome chromosome region 1) gene. The DADA2 phenotype is widely heterogeneous, and patients may present with fever, weight loss, livedo reticularis/racemosa, digital ischemia, cutaneous ulceration, peripheral neuropathy, abdominal pain, bowel perforation, and portal or nephrogenic hypertension. More specific manifestations include early-onset ischemic or hemorrhagic stroke, mild immunodeficiency and hypogammaglobinemia, cytopenia, and vision disturbances. Herein, we present the case of a young male with vasculitis associated with DADA2. The presence of HLA-B51 and the clinical features of this patient raised the question of similarities between ADA2 deficiency, Behçet's disease, and NOD2-associated diseases. Treatment of this rare monogenic disease is challenging and based on small case series. The long-term experience of this patient proved the difficulties of prednisone tapering and the lack of satisfactory therapeutic strategies.


Subject(s)
Behcet Syndrome , Vasculitis , Humans , Male , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Adenosine Deaminase/genetics , Intercellular Signaling Peptides and Proteins/genetics , Vasculitis/etiology
16.
Curr Opin Rheumatol ; 35(5): 278-284, 2023 09 01.
Article in English | MEDLINE | ID: mdl-37433219

ABSTRACT

PURPOSE OF REVIEW: This article serves as an up-to-date examination of the latest findings in the field of paediatric large-vessel and medium-vessel vasculitis. RECENT FINDINGS: Over the last 2 years and in the wake of SARS-CoV2 pandemic, a multitude of studies have increased our insight into these conditions. Although large-vessel and medium-vessel vasculitis are uncommon amongst children, they are a complex and multisystem with a constantly evolving landscape. Increasing numbers of reports from low-income and middle-income countries are shaping our understanding of the epidemiology of vasculitis in children. The influence of infectious disease and the microbiome are of particular interest in unravelling pathogenetic aspects. Improved understanding of the genetics and immunology offer opportunities for better diagnostic options and biomarkers of disease as well as targeted therapies. SUMMARY: In this review, we address recent findings in epidemiology, pathophysiology, clinical findings, bio-markers, imaging and treatment that have the potential to offer better management solutions for these uncommon conditions.


Subject(s)
COVID-19 , Vasculitis , Humans , Child , RNA, Viral , COVID-19/epidemiology , SARS-CoV-2 , Vasculitis/diagnosis , Vasculitis/epidemiology , Vasculitis/etiology , Diagnostic Imaging
17.
Clin Rheumatol ; 42(10): 2761-2775, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37422611

ABSTRACT

The global health crisis caused by the COVID-19 pandemic overwhelmed the capacity of healthcare systems to cope with the rapidly spreading infection and its associated complications. Among these complications, autoimmune phenomena such as systemic vasculitis emerged as a significant challenge. Both the SARS-CoV-2 virus and the vaccines developed to combat it appeared to induce clinical manifestations resembling various types of systemic vasculitis, affecting large, medium, and small vessels. These virus- or vaccine-induced vasculitides exhibited a distinct natural history and course from de novo vasculitis, as they were more responsive to steroid therapy and some mild cases even resolved spontaneously. Notably, there have been no confirmed cases of SARS-CoV-2 infection or vaccination triggering variable vessel vasculitis like Behcet's disease or Kawasaki disease. IgA vasculitis, which is predominantly a pediatric condition, was more prevalent in adults after COVID-19 infection and they had a favorable outcome with glucocorticoid treatment. The impact of immunosuppression, especially B-cell-depleting agents, on the immunogenicity of the vaccine was evident, but there was no significant increase in the incidence of SARS-CoV-2 infection in these patients compared to the general population. Considering their relatively benign course, these post-COVID or post-vaccine vasculitides seem to be amenable to 0.8 to 1 mg/kg prednisolone or equivalent, which could be gradually tapered. The need for immunosuppression and the duration of steroid therapy should be determined on an individual basis. While the world still reels from the perils of a deadly pandemic, the aftermath continues to haunt. Our narrative review aims to explore the effects of COVID and the vaccine on systemic vasculitis, as well as the effect of disease and immunosuppression on the immunogenicity of the COVID vaccine.


Subject(s)
COVID-19 , Systemic Vasculitis , Vasculitis , Adult , Child , Humans , COVID-19 Vaccines/adverse effects , Pandemics , SARS-CoV-2 , Vaccination/adverse effects , Vasculitis/etiology , Phenotype , Steroids
19.
Rheum Dis Clin North Am ; 49(3): 633-645, 2023 08.
Article in English | MEDLINE | ID: mdl-37331737

ABSTRACT

Auricular, nasal, and laryngeal manifestations occur frequently in rheumatic diseases. Inflammatory ear, nose, and throat (ENT) processes often result in organ damage and have profound effects on quality of life. Herein, we review the otologic, nasal, and laryngeal involvement of rheumatic diseases, focusing on their clinical presentation and diagnosis. ENT manifestations generally respond to treatment of the systemic disease, which is outside the scope of this review; however, adjunctive topical and surgical treatment approaches, as well as treatment of idiopathic inflammatory ENT manifestations will be reviewed.


Subject(s)
Autoimmune Diseases , Rheumatic Diseases , Vasculitis , Humans , Pharynx , Quality of Life , Vasculitis/diagnosis , Vasculitis/etiology , Vasculitis/therapy , Autoimmune Diseases/complications , Autoimmune Diseases/diagnosis , Autoimmune Diseases/therapy , Rheumatic Diseases/complications
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