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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 -
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 -
Dermatology Online Journal Feb 2001
Topics: Biopsy; Female; Humans; Middle Aged; Physical Examination; Polyarteritis Nodosa; Prednisone; Skin
PubMed: 11328638
DOI: No ID Found -
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 -
The American Journal of Pathology 1951
Topics: Churg-Strauss Syndrome; Humans; Hypersensitivity; Immune System Diseases; Polyarteritis Nodosa
PubMed: 14819261
DOI: No ID Found -
Medicina (Kaunas, Lithuania) Jun 2023Classic polyarteritis nodosa (PAN) is a vasculitis with systemic manifestations that is characterized by inflammatory and necrotizing lesions affecting medium and small... (Review)
Review
Classic polyarteritis nodosa (PAN) is a vasculitis with systemic manifestations that is characterized by inflammatory and necrotizing lesions affecting medium and small muscular arteries, most frequently at the bifurcation of the vessels. These lesions lead to the formation of microaneurysms, hemorrhaging ruptured aneurysms, thrombosis, and, consequently, ischemia or organ infarction. : We present a complex clinical case of a patient with a late diagnosis of polyarteritis nodosa with multiorgan involvement. : The 44-year-old patient, in an urban environment, presented on her own in the emergency room for acute ischemia phenomena and forearm and right-hand compartment syndrome, requiring surgical decompression in the Plastic Surgery Clinic. : Significant inflammatory syndrome is noted, alongside severe normocytic hypochromic iron deficiency anemia, nitrogen retention syndrome, hyperkalemia, hepatic syndrome, and immunological disturbances: absence of cANCA, pANCA, anti Scl 70 Ac, antinuclear Ac, and anti dDNA Ac, as well as a low C3 fraction of the plasmatic complement system. The morphological aspect described in the right-hand skin biopsy correlated with the clinical data supports the diagnosis of PAN. : The viral form of PAN seems to be individualized as a distinct entity, requiring early, aggressive medication.
Topics: Humans; Female; Adult; Polyarteritis Nodosa; Arteries; Biopsy; Antibodies, Antineutrophil Cytoplasmic
PubMed: 37374366
DOI: 10.3390/medicina59061162 -
Arthritis and Rheumatism Mar 2001
Review
Topics: Churg-Strauss Syndrome; Humans; Polyarteritis Nodosa; Terminology as Topic; Vasculitis
PubMed: 11263763
DOI: 10.1002/1529-0131(200103)44:3<508::AID-ANR96>3.0.CO;2-B -
The British Journal of Dermatology Apr 2002Classic polyarteritis nodosa (PAN) is a segmentary leucocytoclastic vasculitis that affects small- and medium-sized arteries. In 1931, Lindberg (Acta Med Scand 1931; 76:...
Classic polyarteritis nodosa (PAN) is a segmentary leucocytoclastic vasculitis that affects small- and medium-sized arteries. In 1931, Lindberg (Acta Med Scand 1931; 76: 183-225) described the existence of a cutaneous variant of PAN, without visceral involvement and with a more favourable prognosis. We present four patients diagnosed with cutaneous PAN in our hospital between 1987 and 1998. The study group was composed of three women and one child, whose ages ranged from 11 to 70 years old. The follow-up period was between 2 and 13 years. Each patient was submitted for an initial clinical, histological and laboratory evaluation and subsequent follow-up. The presence of nodules was the most frequent cutaneous lesion, preferentially located in the lower limbs. The erythrocyte sedimentation rate was the only parameter that was altered in all patients. Cutaneous biopsies from all patients showed a segmentary leucocytoclastic vasculitis in the arteries of the deep dermis and/or hypodermis. Direct immunofluorescence was positive in just one patient. No visceral involvement was found in any patient. There is confusion about the correct definition of cutaneous PAN. Some clinical findings, such as nodules or livedo reticularis, typical of cutaneous PAN suggest a good prognosis; however, we consider that it is necessary to evaluate these patients for systemic involvement for the possibility of arteritis in other organs as the term polyarteritis suggests.
Topics: Adult; Aged; Child; Female; Follow-Up Studies; Humans; Male; Polyarteritis Nodosa; Prognosis; Retrospective Studies; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 11966708
DOI: 10.1046/j.1365-2133.2002.04624.x -
Arthritis and Rheumatism Oct 2003To describe initial clinical symptoms attributable to microscopic polyangiitis (MPA) or polyarteritis nodosa (PAN).
OBJECTIVE
To describe initial clinical symptoms attributable to microscopic polyangiitis (MPA) or polyarteritis nodosa (PAN).
METHODS
We retrospectively reviewed the medical files of 72 patients (mean followup 6.7 years) with biopsy-proven MPA (n = 36) or PAN (n = 36).
RESULTS
Initial manifestations were similar in both entities except for peripheral neuropathy (P = 0.02) and gastrointestinal tract involvement (P = 0.006), which were significantly more frequent in PAN, and general signs alone in MPA (8%; P = 0.02). The mean time to diagnosis was 9.8 +/- 19.4 months; 35% of the patients died and 26% relapsed; significantly more MPA than PAN patients relapsed (P = 0.03). Time to diagnosis >/=90 days was associated with a trend toward more patients relapsing (P = 0.12), but not with an increased risk of mortality.
CONCLUSION
Initial symptoms of MPA and PAN are usually nonspecific and last for several months before the diagnosis is made. A longer time to diagnosis is associated with a tendency to a higher relapse rate.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Polyarteritis Nodosa; Recurrence; Retrospective Studies; Severity of Illness Index; Survival Rate; Time Factors; Vasculitis
PubMed: 14558058
DOI: 10.1002/art.11387