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Medicine Dec 2023Cutaneous polyarteritis nodosa (cPAN) is a form of medium-sized vessel necrotizing vasculitis. It is a rare, skin-limited variant of polyarteritis nodosa, characterized...
BACKGROUND
Cutaneous polyarteritis nodosa (cPAN) is a form of medium-sized vessel necrotizing vasculitis. It is a rare, skin-limited variant of polyarteritis nodosa, characterized by dermal and subcutaneous tissue involvement. The most common findings in cPAN include digital gangrene, livedo reticularis, and tender subcutaneous nodules. However, while limited to the skin, cPAN results in significant morbidity and mortality due to the accompanying skin ischemia and necrosis, such that patients are vulnerable to superinfection. Here, we describe a unique presentation of cPAN associated with pulmonary arterial hypertension (PAH).
METHODS
A 78-year-old female presented with digital ischemia and leg ulcers associated with PAH. Skin biopsy showed necrotizing fibrinoid necrosis of the small- and middle-sized vessels of the dermis. A diagnosis of cPAN and PAH was made. The patient was treated with glucocorticoids, vasodilators, and cyclophosphamide.
RESULTS
She died due to severe sepsis complications.
CONCLUSION
To date, this is the first case report describing the association between cPAN and PAH. In this case, PAH is a complication of the cutaneous vasculitides suggesting that vasculopathy could play a role in the pathophysiology of PAH. However, the underlying pathophysiological mechanisms still have to be firmly established.
Topics: Female; Humans; Aged; Polyarteritis Nodosa; Pulmonary Arterial Hypertension; Skin Diseases, Vascular; Vasculitis; Necrosis; Familial Primary Pulmonary Hypertension; Ischemia
PubMed: 38115264
DOI: 10.1097/MD.0000000000036563 -
Journal of Vascular Surgery Cases and... Dec 2023Polyarteritis nodosa (PAN) is a rare form of vasculitis. Acute limb ischemia is a rare presentation and complication of PAN. Plain old balloon angioplasty (POBA) is one...
Polyarteritis nodosa (PAN) is a rare form of vasculitis. Acute limb ischemia is a rare presentation and complication of PAN. Plain old balloon angioplasty (POBA) is one of the treatment strategies for addressing PAN-related critical limb threatening ischemia (CLTI). However, recurrence of stenosis and occlusion is frequent, making POBA a poor treatment choice, as evidenced in our described clinical case. Consequently, with consideration of sirolimus's anti-inflammatory and immunosuppressive properties, we used a sirolimus-coated balloon in the treatment of PAN-induced CLTI. A 37-year-old woman first presented with acute limb ischemia as her initial symptom. Diagnostic angiography demonstrated occlusion of her tibial vessels, and POBA was performed to restore perfusion. Later in the course of her illness, she developed foot gangrene despite multiple courses of immunosuppressive drugs and several attempts with POBA to achieve limb salvage. Because of her disease trajectory, a MagicTouch (Concept Medical) sirolimus-coated balloon was deployed to her anterior tibial artery during her third angioplasty. At 17 months after her last angioplasty, she remained ulcer free, and surveillance scans demonstrated occlusion-free tibial vessels. The use of sirolimus-coated balloon angioplasty is a promising treatment approach for successful limb salvage in patients with PAN vasculitis and CLTI.
PubMed: 38106351
DOI: 10.1016/j.jvscit.2023.101266 -
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 -
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 -
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 -
Cureus Sep 2023Polyarteritis nodosa (PAN) is a heterogeneous disease characterized by constitutional symptoms, multi-organ dysfunction, and a subacute to chronic mode of onset. We...
Polyarteritis nodosa (PAN) is a heterogeneous disease characterized by constitutional symptoms, multi-organ dysfunction, and a subacute to chronic mode of onset. We describe a rarely encountered manifestation of PAN in a previously healthy 64-year-old woman who developed acute intra-abdominal bleeding due to multi-vessel, vasculitis-induced retroperitoneal aneurysmal ruptures, followed by the spontaneous cessation of abdominal pain and intra-abdominal bleeding within 24 hours after hospitalization, in the absence of immunosuppressive treatment. Aneurysms regressed and eventually normalized coincident with a moderate-term course of oral corticosteroids and six months of monthly intravenous cyclophosphamide. Our report reveals that patients with life-threatening PAN may present acutely with unexpected historical, physical examination and laboratory features not commensurate with the gravity of the clinical findings. We also highlight that long-term survival lasting greater than 11 years can occur when the correct diagnosis and appropriate treatment are introduced early in the course of the illness.
PubMed: 37868509
DOI: 10.7759/cureus.45718