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Vascular Health and Risk Management 2022Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis affecting small- to medium-sized arteries. The most common gastrointestinal manifestation of PAN is... (Review)
Review
Polyarteritis nodosa (PAN) is a rare systemic necrotizing vasculitis affecting small- to medium-sized arteries. The most common gastrointestinal manifestation of PAN is postprandial abdominal pain from mesenteric arteritis causing bowel ischemia. When transmural ischemia develops, there may be ischemic necrosis and perforation of the bowel wall, which are life-threatening. Severe, life-threatening gastrointestinal involvement is relatively rare in pediatric PAN and may require different management in adult patients. We report a pediatric PAN case in a patient who presented with acute abdominal pain and superimposed cytomegalovirus enteritis with jejunoileal perforation. The patient improved with emergency small intestinal resection followed by conventional immunosuppressive drugs of a corticosteroid and cyclophosphamide, and anti-viral drugs. Before increasing the immunosuppressive drug dosage, initial screening of infectious cytomegalovirus and comprehensive evaluation for surgical conditions are essential in pediatric PAN with severe gastrointestinal involvement. Early aggressive treatment for acute abdomen is useful in reducing morbidity and mortality in pediatric PAN.
Topics: Abdominal Pain; Adult; Child; Cytomegalovirus; Enteritis; Humans; Immunosuppressive Agents; Ischemia; Polyarteritis Nodosa
PubMed: 35924006
DOI: 10.2147/VHRM.S354548 -
CEN Case Reports Nov 2022Polyarteritis nodosa, which is a systemic vasculitis of small- and medium-sized arteries, can cause arterial aneurysms in various organs, sometimes resulting in aneurysm...
Polyarteritis nodosa, which is a systemic vasculitis of small- and medium-sized arteries, can cause arterial aneurysms in various organs, sometimes resulting in aneurysm rupture and hemorrhage. A kidney is one of the major targets of polyarteritis nodosa. Here, we report a 73-year-old woman who presented with sudden-onset high fever, diarrhea, and renal injury with bilateral renal subcapsular hematoma shown on contrast-enhanced computed tomography scan. She did not have trauma and significant medical history other than breast cancer in remission. Serological and immunological tests except for anti-Sjögren's syndrome-A and anti-Sjögren's syndrome-B were all negative. Digital subtraction angiography revealed bilateral intrarenal micro aneurysms, which allowed us to diagnose the patient with polyarteritis nodosa. As continuous monitoring of bilateral intrarenal hematoma by ultrasonography and computed tomography scan did not detect progression of intrarenal hemorrhage and extra renal hematoma, transcatheter arterial embolization and nephrectomy were not performed. Although hemodialysis therapy was required temporarily for acute kidney injury with anuria, her general condition and kidney function remarkably improved after receiving systemic immunosuppressive therapy with corticosteroids and cyclophosphamide. In conclusion, this is a rare case of polyarteritis nodosa manifesting as spontaneous bilateral subcapsular renal hemorrhage with deteriorated renal function, which was successfully treated with immunosuppressive therapy.
Topics: Female; Humans; Aged; Polyarteritis Nodosa; Hematoma; Kidney; Aneurysm; Hemorrhage; Acute Kidney Injury
PubMed: 35171449
DOI: 10.1007/s13730-022-00691-5 -
Pediatric Rheumatology Online Journal Dec 2021Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and... (Review)
Review
BACKGROUND
Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and refractory to standard treatment including prednisone, azathioprine and cyclophosphamide.
CASE PRESENTATION
We present the case of a young girl with severe PAN resulting in progressive ischemia and necrosis of fingers and toes. Biological work-up revealed increased acute phase reactants and interleukin-6 levels. She was only partially controlled despite high-dose corticosteroids and cyclophosphamide infusions, and eventually achieved rapid improvement and sustained remission on tocilizumab. Further, we review the current evidence of the interleukin-6-inhibitor tocilizumab for the treatment of PAN.
CONCLUSION
Tocilizumab may be an efficient therapeutic option in a subset of treatment-refractory children with PAN.
Topics: Antibodies, Monoclonal, Humanized; Child, Preschool; Female; Foot; Hand; Humans; Polyarteritis Nodosa
PubMed: 34861842
DOI: 10.1186/s12969-021-00654-7 -
Internal Medicine (Tokyo, Japan) Jan 2018Polyarteritis nodosa (PAN) is a medium vessel vasculitis affecting systemic organs. Muscle involvement of PAN usually lacks elevation of creatinine kinase (CK). We...
Polyarteritis nodosa (PAN) is a medium vessel vasculitis affecting systemic organs. Muscle involvement of PAN usually lacks elevation of creatinine kinase (CK). We herein report a case of PAN with rhabdomyolysis. A 71-year-old man was hospitalized because of muscle weakness of the lower limbs that persisted for 1 month. On a physical examination, rapidly progressive lower proximal muscle weakness and bilateral drop foot were observed. His blood test showed an elevation in the C-reactive protein (19.5 mg/dL) and CK (13,435 IU/L) levels and negativity for anti-neutrophilic cytoplasmic antibody. Computed tomographic angiography showed stenosis of the left renal artery. Electromyogram indicated mono-neuritis multiplex pattern, and enhanced magnetic resonance imaging demonstrated discretely granular hyperintensities on T2 and slow tau inversion recovery in his femoral muscles. A femoral muscle-biopsy specimen showed fibrinoid necrosis of medium-sized vessels and disruption of the elastic lamina of the vessel wall in fascia. Furthermore, muscle necrosis was localized depending on the arterial distribution, suggesting ischemic changes in the muscles. Given these findings, he was diagnosed with PAN with rhabdomyolysis and treated with methyl-prednisolone pulse therapy followed by oral prednisolone at 50 mg/day. He was additionally treated with monthly intravenous cyclophosphamide at 500 mg. Sustained remission has been obtained for two months since the treatment. Although rhabdomyolysis rarely manifests with PAN, it should be included in a differential diagnosis of febrile patients presenting with acute myalgia and weakness with CK elevation.
Topics: Aged; Anti-Inflammatory Agents; Antibodies, Antineutrophil Cytoplasmic; Antirheumatic Agents; Cyclophosphamide; Humans; Male; Muscle Weakness; Polyarteritis Nodosa; Prednisolone; Rhabdomyolysis; Treatment Outcome
PubMed: 29021478
DOI: 10.2169/internalmedicine.8913-17 -
Clinical and Experimental Immunology Jul 1995
Review
Topics: Antibodies, Antineutrophil Cytoplasmic; Autoantibodies; Autoimmune Diseases; Blood Vessels; Diagnosis, Differential; Humans; Kidney; Liver; Lung; Polyarteritis Nodosa; Vasculitis
PubMed: 7606855
DOI: 10.1111/j.1365-2249.1995.tb06157.x -
The Canadian Journal of Cardiology Jun 2021Coronary artery involvement is a severe but uncommon manifestation of polyarteritis nodosa (PAN), so clinicians have little knowledge of it. Our aim was to investigate...
BACKGROUND
Coronary artery involvement is a severe but uncommon manifestation of polyarteritis nodosa (PAN), so clinicians have little knowledge of it. Our aim was to investigate the clinical characteristics, risk factors and outcomes of patients with PAN complicated with coronary artery lesions.
METHODS
Data from 145 patients with PAN who were admitted to Peking Union Medical College Hospital from January 2000 to September 2019 were retrospectively collected.
RESULTS
Nineteen patients (13.1%) had coronary artery lesions due to PAN. The age at the onset of PAN was 32.3 ± 11.8 years. There were no significant differences in common risk factors for coronary arterial atherosclerosis between the patients with coronary artery involvement and those without. Affected branches of the coronary arteries were left anterior descending branch (15 patients), right coronary artery (14 patients), and left circumflex branch (9 patients). Eleven of the 19 patients exhibited multivessel lesions. Multivariate logistic regression analysis showed that celiac artery involvement (odds ratio [OR] 3.722, 95% confidence interval [CI] 1.115-12.427; P = 0.033) and new-onset hypertension (OR 6.668, 95% CI 1.936-22.961; P = 0.003) were risk factors for coronary artery involvement in patients with PAN. Stent placement was performed for 2 patients, and in-stent restenosis occurred in 1 of those patients a year later.
CONCLUSIONS
PAN with coronary artery involvement exhibits more combined involvement of arteries of other organs and more severe diseases. PAN should be considered when treating young adults with an unknown origin of coronary artery lesions. In addition to systemic immunosuppressive treatment, other measures including antiplatelet and anticoagulation therapy should be initiated; however, determining the optimal time to perform procedures such as intervention or surgery is still challenging.
Topics: Adult; Celiac Artery; China; Coronary Artery Disease; Female; Humans; Hypertension; Immunosuppressive Agents; Male; Percutaneous Coronary Intervention; Platelet Aggregation Inhibitors; Polyarteritis Nodosa; Risk Factors; Severity of Illness Index; Stents; Time-to-Treatment
PubMed: 33310144
DOI: 10.1016/j.cjca.2020.11.011 -
Internal Medicine (Tokyo, Japan) Jan 1994We report localized polyarteritis nodosa in a 31-year-old man who had painful nodules in the left forearm and scrotum. Histopathological findings of both tissues... (Review)
Review
We report localized polyarteritis nodosa in a 31-year-old man who had painful nodules in the left forearm and scrotum. Histopathological findings of both tissues revealed distinct arteritis. However, he had no clinical evidence of any systemic disease. We finally diagnosed this case as a localized polyarteritis nodosa occurring in both the left forearm and epididymis. This form of polyarteritis nodosa has not been reported in the literature.
Topics: Adult; Diagnosis, Differential; Epididymis; Forearm; Humans; Male; Polyarteritis Nodosa; Testicular Neoplasms
PubMed: 7910055
DOI: 10.2169/internalmedicine.33.48 -
Reumatologia Clinica Feb 2022Polyarteritis nodosa (PAN) is a rare form of vasculitis which involves medium and small sized arteries. It can involve any system of the body, but lungs are typically...
Polyarteritis nodosa (PAN) is a rare form of vasculitis which involves medium and small sized arteries. It can involve any system of the body, but lungs are typically spared. PAN typically presents with non-specific symptoms, such as fever, weight loss and myalgias, and symptoms related to the system involved. Negative serum ANCA helps in differentiating it from other similarly presenting vasculitis and definitive diagnosis is made on histopathology. Limb ischaemia is a rare presentation as well as a rare complication of PAN. We present the case of a 28-year-old female who presented with severe lower limb ischaemia and intermittent leg claudication as initial symptoms which led to initial diagnosis of critical limb ischaemia. Later in the course of the illness she developed gastrointestinal symptoms and weight loss. She was diagnosed as having PAN based on laboratory results and histopathological evidence. Intravenous steroids halted the progress of the disease, but the patient required transmetatarsal amputation of the foot to remove dead tissue. Vasculitis like PAN, although much rarer than atherosclerotic peripheral vascular disease (PVD), should be considered as a differential diagnosis in cases of limb ischaemia in which there is lack of risk factors for PVD.
Topics: Adult; Diagnosis, Differential; Female; Humans; Ischemia; Polyarteritis Nodosa; Vasculitis
PubMed: 35153035
DOI: 10.1016/j.reumae.2020.08.010 -
Internal Medicine (Tokyo, Japan) Jan 2022A 23-year-old man presented with severe hypertension. Based on his history of minocycline treatment for over three years and clinical symptoms, such as myalgias and... (Review)
Review
A 23-year-old man presented with severe hypertension. Based on his history of minocycline treatment for over three years and clinical symptoms, such as myalgias and renovascular hypertension with multiple intrarenal aneurysms, he was diagnosed with minocycline-induced renal polyarteritis nodosa (PAN). After minocycline treatment cessation and management of the hypertension, his blood pressure, renin-aldosterone levels, and urinary protein levels gradually improved. Seven and a half years later, repeated angiography found that the aneurysms had resolved. This is the first report in English describing a case of minocycline-induced renal PAN that was reversed functionally and morphologically without steroids or immunosuppressive drugs.
Topics: Adult; Aneurysm; Humans; Hypertension, Renovascular; Kidney; Male; Minocycline; Polyarteritis Nodosa; Young Adult
PubMed: 34176836
DOI: 10.2169/internalmedicine.7340-21 -
The Israel Medical Association Journal... Aug 2019
Topics: Aged; Antibodies, Monoclonal, Humanized; Drug Therapy, Combination; Humans; Male; Polyarteritis Nodosa; Receptors, Interleukin-6
PubMed: 31474022
DOI: No ID Found