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International Journal of Molecular... Nov 2023Polyarteritis nodosa (PAN), also known as panarteritis nodosa, represents a form of necrotizing vasculitis that predominantly affects medium-sized vessels, although it... (Review)
Review
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. Orchitis is a rare but specific manifestation of PAN. The absence of granulomas, glomerulonephritis, and anti-neutrophil cytoplasmic antibodies serves to distinguish PAN from other types of vasculitis. Major complications consist of hemorrhagic and thrombotic events occurring in mesenteric, cardiac, cerebral, and renal systems. Historically, PAN was frequently linked to hepatitis B virus (HBV) infection, but this association has dramatically changed in recent years due to declining HBV prevalence. Current epidemiological research often identifies a connection between PAN and genetic syndromes as well as neoplasia. This article provides a comprehensive review of PAN, specifically focusing on the progression of its clinical manifestations over time.
Topics: Male; Humans; Polyarteritis Nodosa; Vasculitis; Hepatitis B; Hepatitis B virus; Gastrointestinal Tract
PubMed: 38068989
DOI: 10.3390/ijms242316668 -
Rheumatology (Oxford, England) May 2024
Topics: Humans; Polyarteritis Nodosa; Positron Emission Tomography Computed Tomography; Fluorodeoxyglucose F18; Early Diagnosis; Radiopharmaceuticals; Female; Male; Middle Aged
PubMed: 38058205
DOI: 10.1093/rheumatology/kead655 -
Cureus Oct 2023Polyarteritis 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...
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. It demonstrated inflammatory demyelinating disease of the central nervous system or multiple cerebral infarctions attributable to vasculitis, and it is difficult to differentiate between them based on image findings alone, and cannot be determined without following the clinical course. Hence, we treated with steroid therapy, which is effective for both conditions. Although the paralysis was alleviated, an MRI of the brain reperformed on day 7 revealed expansion of the lesion with contrast enhancement in the feeding area of the left lateral striatal artery, a high signal in DWI, and a low signal in an ADC map. Based on the clinical and radiological findings, we diagnosed a cerebral infarction attributable to vasculitis. Contrast computed tomography (CT) of her chest and abdominal CT angiography revealed that she met the diagnostic criteria for PAN, and adenosine deaminase 2 (AD2) activity level was low. The patient was treated with steroids combined with azathioprine and cyclophosphamide but three weeks after discharge developed a new cerebral infarction in the right basal ganglia. We commenced infliximab; no recurrence of cerebral infarction has been noted. The low AD2 activity may explain the intractable atypical course of this case. Further studies are needed to reveal the role of AD2 in patients with residual enzyme activity and reevaluation of the PAN diagnostic criteria is essential.
PubMed: 38034163
DOI: 10.7759/cureus.47952 -
Cureus Nov 2023Polyarteritis nodosa (PAN) is a rare autoimmune vasculitis characterized by the inflammation of medium-sized arteries throughout the body. This case report presents the...
Polyarteritis nodosa (PAN) is a rare autoimmune vasculitis characterized by the inflammation of medium-sized arteries throughout the body. This case report presents the clinical course of a 48-year-old female patient who experienced a complex diagnostic journey and complications during the management of PAN. The patient initially presented with dry skin, rash, and pruritus, which led to an extensive evaluation. Despite multiple visits and investigations, the definitive diagnosis of PAN was delayed. Eventually, the patient was diagnosed with PAN based on skin biopsy findings demonstrating vasculitis and inflammation of blood vessel walls. The treatment course was further complicated by the development of steroid-induced diabetes and recurrent vasculitis symptoms. Recurrence of symptoms, including rashes and neuropathy, necessitated adjustments in therapeutic interventions. This case highlights the challenges encountered in managing both PAN and its associated complications and emphasizes the importance of a multidisciplinary approach and improved patient compliance to optimize treatment outcomes.
PubMed: 38033446
DOI: 10.7759/cureus.49677 -
Journal of Surgical Case Reports Nov 2023Medium and small arteries are mainly affected by polyarteritis nodosa. Lungs are spared but any other organ can be involved. Gallbladder can be part of this systemic...
Medium and small arteries are mainly affected by polyarteritis nodosa. Lungs are spared but any other organ can be involved. Gallbladder can be part of this systemic disease. Isolated gallbladder disease is not common. The presentation of the systemic polyarteritis nodosa as acute cholecystitis is described in this case report. Management of the disease depends on the involved organs and usually consists of systemic steroids. The diagnosis of polyarteritis nodosa should be considered in patients with previous systemic symptoms who develop picture of acute cholecystitis.
PubMed: 38026741
DOI: 10.1093/jscr/rjad603 -
Scientific Reports Nov 2023To save time during transport, where resuscitation quality can degrade in a moving ambulance, it would be prudent to continue the resuscitation on scene if there is a...
To save time during transport, where resuscitation quality can degrade in a moving ambulance, it would be prudent to continue the resuscitation on scene if there is a high likelihood of ROSC occurring at the scene. We developed the pre-hospital real-time cardiac arrest outcome prediction (PReCAP) model to predict ROSC at the scene using prehospital input variables with time-adaptive cohort. The patient survival at discharge from the emergency department (ED), the 30-day survival rate, and the final Cerebral Performance Category (CPC) were secondary prediction outcomes in this study. The Pan-Asian Resuscitation Outcome Study (PAROS) database, which includes out-of-hospital cardiac arrest (OHCA) patients transferred by emergency medical service in Asia between 2009 and 2018, was utilized for this study. From the variables available in the PAROS database, we selected relevant variables to predict OHCA outcomes. Light gradient-boosting machine (LightGBM) was used to build the PReCAP model. Between 2009 and 2018, 157,654 patients in the PAROS database were enrolled in our study. In terms of prediction of ROSC on scene, the PReCAP had an AUROC score between 0.85 and 0.87. The PReCAP had an AUROC score between 0.91 and 0.93 for predicting survived to discharge from ED, and an AUROC score between 0.80 and 0.86 for predicting the 30-day survival. The PReCAP predicted CPC with an AUROC score ranging from 0.84 to 0.91. The feature importance differed with time in the PReCAP model prediction of ROSC on scene. Using the PAROS database, PReCAP predicted ROSC on scene, survival to discharge from ED, 30-day survival, and CPC for each minute with an AUROC score ranging from 0.8 to 0.93. As this model used a multi-national database, it might be applicable for a variety of environments and populations.
Topics: Humans; Cardiopulmonary Resuscitation; Emergency Medical Services; Out-of-Hospital Cardiac Arrest; Hospitals; Outcome Assessment, Health Care; Polyarteritis Nodosa
PubMed: 37990066
DOI: 10.1038/s41598-023-45767-z -
Journal of Clinical Medicine Oct 2023Ocular manifestations have been described in the course of various types of vasculitis. However, there seems to be no routine ophthalmological examinations for patients...
Evaluation of Choroidal and Retinal Features in Patients with Primary Vasculitis-An Original Optical Coherence Tomography and Optical Coherence Tomography Angiography Study.
Ocular manifestations have been described in the course of various types of vasculitis. However, there seems to be no routine ophthalmological examinations for patients suffering from those diseases. To ensure holistic care we aimed to investigate any retinal and choroidal abnormalities in patients suffering from primary vasculitis. The objective was to use non-invasive methods, which would not be time- and cost-consuming, yet would be helpful in routine tests. We conducted a prospective and observational study in 41 patients (78 eyes) with 5 types of primary vasculitis, including: Takayasu's arteritis; giant cell arteritis; Buerger's disease; granulomatosis with polyangiitis; and polyarteritis nodosa. A total of 44 healthy individuals were enrolled in the control group for comparison (88 eyes). With the use of optical coherence tomography, optical coherence tomography angiography, and MATLAB, the following parameters were assessed: choroidal thickness; vascularity index; area and perimeter of foveal avascular zone; and circularity index. The following parameters were lower in the study group compared to the control group: mean nasal and temporal CTs; mean central, temporal, and nasal CVI; and mean CI. In contrast, the results of mean central CT as well as the area and perimeter of FAZ were higher in the study group. The differences were statistically significant in the case of all parameters except for CI. Conducting routine ophthalmological examinations in patients diagnosed with vasculitis by assessment of the retina and choroid by measuring parameters like CT, CVI, area and perimeter of FAZ, and CI could be beneficial, as it may detect pathological changes before any ocular symptoms alarm the patients. CVI seems to be especially promising for choroidal evaluation, as it appears to be less influenced by various factors compared to CT.
PubMed: 37959292
DOI: 10.3390/jcm12216827 -
The Journal of Dermatology Feb 2024Autoinflammatory diseases (AIDs) characterized by recurrent episodes of localized or systemic inflammation are disorders of the innate immune system. Skin lesions are... (Review)
Review
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.
Topics: Adult; Humans; Child; Middle Aged; Adenosine Deaminase; Livedo Reticularis; Intercellular Signaling Peptides and Proteins; Vasculitis; Polyarteritis Nodosa; Skin Diseases; Familial Mediterranean Fever; Mutation; Agammaglobulinemia; Myelodysplastic Syndromes; Skin Diseases, Genetic; Severe Combined Immunodeficiency
PubMed: 37955334
DOI: 10.1111/1346-8138.17030 -
Pediatric Rheumatology Online Journal Nov 2023Rheumatic patients have a higher frequency of tuberculosis(TB) than the general population. This study aimed to describe children and adolescents with TB and rheumatic...
BACKGROUND
Rheumatic patients have a higher frequency of tuberculosis(TB) than the general population. This study aimed to describe children and adolescents with TB and rheumatic diseases(RD) who were being treated in a reference center.
METHODS
A series of TB cases were investigated in a reference center for childhood TB in Rio de Janeiro, Brazil, from 1995 to 2022.
RESULTS
Fifteen patients with underlying RD and TB were included with 8(53%) being female. The mean age at RD diagnosis was 7.10years (SD ± 0,57 years), and the mean age at TB diagnosis was 9.81 years(SD ± 0.88 years). A total of 9 cases of pulmonary TB(PTB) and 6 cases of extrapulmonary TB-pleural(2), joint/osteoarticular(1), cutaneous(1), ocular(1), and peritoneal(1)- were described. The RD observed in the 15 patients included juvenile idiopathic arthritis(9), juvenile systemic lupus erythematosus(3), juvenile dermatomyositis(1), polyarteritis nodosa(1), and pyoderma gangrenosum(1). Among the immunosuppressants/immunobiologics, methotrexate(8) was the most commonly used, followed by corticosteroids(6), etanercept(2), mycophenolate mofetil(1), cyclosporine A(1), adalimumab(1), and tocilizumab(1). The most common symptoms were fever and weight loss, and a predominance of PTB cases was noted. GeneXpert MTB/RIF® was performed in six patients and was detectable in two without rifampicin resistance; Xpert Ultra® was performed in five patients, and traces with indeterminate rifampicin resistance were detected in three. One female patient discontinued treatment, and another passed away.
CONCLUSIONS
The case series demonstrated the importance of suspecting and investigating TB in RD affected patients who are using immunosuppressants/ immunobiologics, particularly in countries with high rates of TB such as Brazil.
Topics: Humans; Child; Female; Adolescent; Male; Rifampin; Mycobacterium tuberculosis; Sensitivity and Specificity; Brazil; Tuberculosis; Rheumatic Diseases; Immunosuppressive Agents
PubMed: 37950309
DOI: 10.1186/s12969-023-00918-4 -
Modern Rheumatology Case Reports Dec 2023Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis with a poor prognosis, characterised by inflammation and necrosis of medium-sized arteries. PAN patients...
Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis with a poor prognosis, characterised 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 favourable 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. Although contrast-enhanced computed tomography and computed tomographic angiography are increasingly used to diagnose PAN, neither case had any abnormal findings on these examinations. Our cases therefore underscore that catheter-based angiography is critical for differentiation between PAN and CA, even in cases with limited systemic symptoms.
Topics: Humans; Polyarteritis Nodosa; Arteritis; Vasculitis; Skin; Angiography
PubMed: 37947049
DOI: 10.1093/mrcr/rxad064