Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa

Hočevar, Alojzija; Tomšič, Matija; Pirkmajer, Katja Perdan

doi:10.1007/s11926-021-00983-2

Cited by 34 publications

(34 citation statements)

References 91 publications

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“…PAN is a systemic medium-vessel necrotizing vasculitis that most frequently involves the skin, kidneys, and peripheral nervous system and is diagnosed by a combination of clinical, angiographic, and pathologic findings [ 2 ]. Cardiovascular involvement occurs in only 5-22% of patients and carries a two to threefold higher mortality compared to those without coronary involvement [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Cardiovascular involvement occurs in only 5-22% of patients and carries a two to threefold higher mortality compared to those without coronary involvement [ 3 ]. Coronary involvement in PAN is angiographically characterized by alternating areas of stenosis or occlusion with aneurysm in a “beads on a string” pattern [ 1 , 2 ]. Treatment involves aggressive immunosuppression, typically with corticosteroids and cyclophosphamide [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

“…Coronary involvement in PAN is angiographically characterized by alternating areas of stenosis or occlusion with aneurysm in a “beads on a string” pattern [ 1 , 2 ]. Treatment involves aggressive immunosuppression, typically with corticosteroids and cyclophosphamide [ 2 ]. DADA2 is an autoinflammatory disorder first described in 2014 that clinically resembles PAN and is characterized by vasculitis, dysregulated immune function, and hematologic abnormalities [ 4 , 5 ].…”

Section: Discussionmentioning

confidence: 99%

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Fulminant Vasculitis Complicated by ST-Elevation Myocardial Infarction and Stroke

Pham

Kovach

Patti

et al. 2021

Case Reports in Cardiology

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Acute coronary syndrome is a rare complication of vasculitis. We present a case of fulminant medium-vessel vasculitis, most likely PAN, complicated by STEMI and stroke, that was successfully treated with percutaneous revascularization, high-quality stroke care, and immunosuppression. This case highlights the importance of prompt diagnosis and treatment of vasculitis and the recognition of coronary and cerebral ischemia as potentially serious complications.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Fulminant Vasculitis Complicated by ST-Elevation Myocardial Infarction and Stroke

Pham

Kovach

Patti

et al. 2021

Case Reports in Cardiology

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“…It is present in 1–5% of the cases of HBV infections, while 35–50% of the patients with PAN are positive for HBsAg [ 32 , 33 ]. Notably, the prevalence of HBV-related PAN appears to have fallen to less than 5% with the introduction of the HBV vaccine [ 34 ]. The precipitation of circulating immune complexes containing an excess of HBV antigens seems to be one of the pathogenic mechanisms underlying PAN [ 35 ].…”

Section: Hbv and Rheumatologic Manifestationsmentioning

confidence: 99%

“…The diagnostic hallmark of the disease is the detection of multiple aneurisms in the affected organs. Ischemic or hemorrhagic sequelaes with infarction or hematomas are common [ 34 ].…”

Section: Hbv and Rheumatologic Manifestationsmentioning

confidence: 99%

Extrahepatic Manifestations of Chronic HBV Infection and the Role of Antiviral Therapy

Mazzaro

Adinolfi

Pozzato

et al. 2022

JCM

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The hepatitis B virus (HBV) infection leads to chronic hepatitis, cirrhosis, and hepatocarcinoma. However, about 20% of patients experience extrahepatic manifestations such as polyarteritis nodosa, non-rheumatoid arthritis, non-Hodgkin lymphoma, cryoglobulinemic vasculitis, and glomerulonephritis. These influence the patient’s morbidity, quality of life and mortality. The treatment of an HBV infection is based on nucleotide analogues (NAs) which are safe and effective for the suppression of HBV-DNA in almost 100% of cases. A few studies have shown that NAs induce a viral response and an improvement of extrahepatic diseases. There is a lack of a thorough analysis of the available treatments for extrahepatic HBV manifestations. In 90% to 100% of cases, the NAs stop the HBV replication, and they produce a clinical response in the majority of patients with mild to moderate extrahepatic signs/symptoms. Arthritis can definitely disappear after the HBV elimination and, in some cases, the HBV eradication following NAs therapy appears to improve the renal function in HBV-related nephropathies. Plasma exchange can be used in subjects who are suffering from the most aggressive forms of cryoglobulinemic vasculitis and glomerulonephritis, progressive peripheral neuropathy, and life-threatening cases, and this can be combined with glucocorticosteroids and antiviral agents. In selected refractory patients, the use of rituximab in conjunction with NAs therapy can be considered. The review provides an update on extrahepatic conditions that are linked to HBV and the impact of treating HBV with NAs.

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Case report of streptococcal infection as a potential precipitating factor in cutaneous polyarteritis nodosa in pediatric patients

Tabibi,

Shiari,

Shafiee

et al. 2024

Clinical Case Reports

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Key Clinical MessageThis pediatric case report underscores the importance of maintaining a high clinical suspicion for polyarteritis nodosa (PAN) in patients presenting with atypical features, such as migratory arthritis and subcutaneous nodules. Importantly, it highlights the focus on the potential relationship between streptococcal infection and cutaneous PAN. Early recognition and prompt, aggressive treatment is critical, as PAN can be a life‐threatening condition if left unmanaged. This case emphasizes the need for a multidisciplinary approach to effectively identify and manage this rare vasculitis disorder in the pediatric population.AbstractPolyarteritis nodosa (PAN) is a rare and life‐threatening vasculitis with diverse clinical presentations, posing a diagnostic challenge. Early recognition and prompt intervention are crucial to prevent organ damage. We present the case of an 8‐year‐old boy who exhibited atypical symptoms including migratory arthritis, myalgia, digital discoloration and ischemic changes, and subcutaneous nodules. Initial concerns for septic arthritis were ruled out. A comprehensive evaluation revealed elevated inflammatory markers and a confirmatory skin biopsy demonstrating active leukocytoclastic vasculitis, are highly suggestive of a diagnosis of PAN. Notably, elevated ASO titers suggested a possible concurrent streptococcal infection. The aggressive treatment approach with high‐dose aspirin, steroids, methotrexate, and tocilizumab is justified given the severity of the patient's symptoms and the nature of the disease process. This case underscores the importance of considering PAN in the differential diagnosis for children presenting with atypical features. Early diagnosis and prompt intervention, including addressing potential infectious triggers, are crucial for optimal outcomes in pediatric PAN.

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Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa

Cited by 34 publications

References 91 publications

Fulminant Vasculitis Complicated by ST-Elevation Myocardial Infarction and Stroke

Fulminant Vasculitis Complicated by ST-Elevation Myocardial Infarction and Stroke

Extrahepatic Manifestations of Chronic HBV Infection and the Role of Antiviral Therapy

Case report of streptococcal infection as a potential precipitating factor in cutaneous polyarteritis nodosa in pediatric patients

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