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Autoimmunity Reviews Jul 2018The aim of the study was to describe the evolution of mortality and cause-specific mortality over time in patients with systemic necrotizing vasculitides (SNV),... (Review)
Review
OBJECTIVE
The aim of the study was to describe the evolution of mortality and cause-specific mortality over time in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).
METHODS
Patients with SNV from the French Vasculitis Study Group registry were divided into 5 groups according to the date of diagnosis: <1980, 1980-1989, 1990-1999, 2000-2010, and ≥ 2010. The causes of death were classified as vasculitis, infection, cardiovascular, malignancy, miscellaneous, or unknown.
RESULTS
Among the 2217 patients included (PAN 16.1%, GPA 41.7%, EGPA 22.6%, MPA 19.6%), overall incidence of death was 2.26 per 100 person-years. The overall survival improved during each period considered. The 5-year survival rate increased from 72.2% (95% confidence interval [CI] 59.7-87.2) for patients diagnosed before 1980 to 94.5% (95% CI 90.4-98.8) after 2010 (p < 0.001). Periods of diagnosis, age, and male gender were independently associated with a poor survival with a non-significant difference between vasculitis. The incidence of mortality between the 1980s and after 2010 significantly decreased for vasculitis-related (p = 0.03) and cardiovascular-related deaths (p = 0.04). Incidence of death by infection remained stable between the 1980s and the 2000s but no death by infection occurred after 2010. The incidence of death by malignancy remained stable over time.
CONCLUSION
Overall survival of SNV patients has improved since the 1980s with the decrease of vasculitis- and cardiovascular-related deaths, but cancer-related mortality remained stable. These results highlight malignancy as the current target to improve the overall prognosis.
Topics: France; Humans; Incidence; Polyarteritis Nodosa; Prognosis; Registries; Retrospective Studies; Survival Rate
PubMed: 29730524
DOI: 10.1016/j.autrev.2018.01.022 -
Journal of Comparative Pathology Feb 2019An 18-month-old neutered male domestic shorthair cat was referred with a history of pyrexia, polyuria and polydypsia, and transient episodes of bilateral hindlimb...
An 18-month-old neutered male domestic shorthair cat was referred with a history of pyrexia, polyuria and polydypsia, and transient episodes of bilateral hindlimb paralysis. Cardiac evaluation revealed severe systemic hypertension and severe concentric hypertrophy of the left ventricle. One month later the cat had a new episode of hindlimb paralysis with recurrent seizures, and died in status epilepticus. At necropsy examination, the coronary arteries, arcuate renal arteries and common iliac arteries showed marked thickening with nodules segmentally located along the vessels and consequent narrowing of the lumina. Histologically, acute and chronic inflammatory infiltration of the vascular walls was associated with necrosis of the muscular layer. Lesions were consistent with polyarteritis nodosa and involved the small, medium and large arteries of the heart, kidneys, small and large intestine, mesentery, liver and thyroid. Multifocal meningeal vasculitis associated with focal infarction of the frontal lobe and necrotizing vascular polyneuropathy were detected. Involvement of the central and peripheral nervous systems in polyarteritis nodosa is a novel finding in cats.
Topics: Animals; Cat Diseases; Cats; Central Nervous System; Male; Peripheral Nervous System; Polyarteritis Nodosa
PubMed: 30898299
DOI: 10.1016/j.jcpa.2018.11.003 -
Journal of Medical Case Reports Jul 2019Polyarteritis nodosa is a small vessel to medium vessel vasculitis that frequently presents with multi-organ involvement, but can sometimes be limited to single organs... (Review)
Review
BACKGROUND
Polyarteritis nodosa is a small vessel to medium vessel vasculitis that frequently presents with multi-organ involvement, but can sometimes be limited to single organs such as the testes. Patients often require treatment with glucocorticoids, plus or minus additional immunosuppressive therapy depending on the severity of the disease. We describe a rare case of polyarteritis nodosa involving the right testis and urinary bladder without other systemic features of vasculitis.
CASE PRESENTATION
A previously healthy 54-year-old First Nations Canadian man presented with intermittent gross hematuria. He underwent surgical excision of his right testis for cryptorchidism and a transurethral resection of a bladder mass. Histology showed an active medium vessel vasculitis in both organs. On extensive clinical, laboratory, and radiographic review, he had no systemic features of vasculitis. On 2-year follow-up, he has not required any systemic therapy and has not developed further symptoms.
CONCLUSION
Single organ polyarteritis nodosa can sometimes be managed with surgical excision of the involved organ alone. Although our patient had two organs involved, we extrapolated the results of our literature search to guide his care. This case highlights the potential for surgical excision to cure polyarteritis nodosa despite the involvement of two organs in the absence of symptoms and signs of systemic vasculitis.
Topics: Cryptorchidism; Humans; Male; Middle Aged; Orchiectomy; Polyarteritis Nodosa; Testis; Urinary Bladder; Urinary Bladder Diseases
PubMed: 31362782
DOI: 10.1186/s13256-019-2172-y -
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 -
British Medical Journal Mar 1969
Topics: Arthritis, Rheumatoid; Humans; Kidney Diseases; Lung Diseases; Lupus Erythematosus, Systemic; Neurologic Manifestations; Polyarteritis Nodosa; Prognosis; Skin Manifestations
PubMed: 4388140
DOI: 10.1136/bmj.1.5647.827 -
British Medical Journal Mar 1973
Topics: Adult; Humans; Male; Neurologic Manifestations; Peripheral Nervous System Diseases; Polyarteritis Nodosa; Skin Diseases; Skin Manifestations
PubMed: 4144244
DOI: 10.1136/bmj.1.5852.551 -
British Medical Journal Jan 1973
Topics: Adrenal Cortex Hormones; Humans; Polyarteritis Nodosa; Prognosis; Skin Diseases
PubMed: 4405238
DOI: No ID Found -
Proceedings of the Royal Society of... Aug 1970
Topics: Arteritis; Erythema Nodosum; Humans; Hypersensitivity; Polyarteritis Nodosa; Purpura; Skin Diseases; Vascular Diseases
PubMed: 4393789
DOI: No ID Found -
The American Journal of Pathology Jul 1948
Topics: Blood Vessels; Cell Differentiation; Humans; Hypersensitivity; Polyarteritis Nodosa
PubMed: 18874416
DOI: No ID Found -
Edinburgh Medical Journal Mar 1947
Topics: Humans; Polyarteritis Nodosa
PubMed: 20241006
DOI: No ID Found