Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda.

Polyarteritis nodosa (PAN) is a rare form of primary systemic vasculitis with heterogeneous presentations, treatments and disease course. Historical approaches to classification and diagnostic terminology are reviewed. Since differentiation of PAN from microscopic polyangiitis (MPA) and other ANCA vasculitides by the Chapel Hill conference statements, and with hepatitis associated PAN defined as a secondary vasculitis, the phenotyping and subclassification of PAN has received little attention. Monogenic disorders similar to PAN have been described (familial Mediterranean fever, Adenosine Deaminase-2 deficiency), and cutaneous PAN and single organ vasculitis, discussed. The overlapping phenotypes between PAN and other primary vasculitic syndromes and subphenotypes within PAN are explored. This work will underpin development of newer treatment regimens and future genetic and related aetiologic studies.

Perspectiva histórica de la clasificación de las vasculitis / Historical perspective on the classification of vasculitis

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Nosology of primary vasculitis.

PURPOSE OF REVIEW: Recent developments in the nosology of primary systemic vasculitis are placed in the context of an historical overview. The ongoing attempts to develop criteria for classification and diagnosis are discussed. RECENT FINDINGS: Giant cell arteritis has supplanted temporal arteritis as the preferred term for chronic granulomatous arteritis in older adults. A new classification system for childhood vasculitis has been proposed by a European collaborative group. A study of idiopathic polyarteritis nodosa demonstrates greater similarity to microscopic polyangiitis in relapse rate than previously reported. Controversy has arisen over the eponym Wegener's granulomatosis because of alleged involvement of Friedrich Wegener in the Nazi regime during World War II. Diagnostic criteria for Kawasaki disease are problematic because many patients with coronary artery involvement do not fulfill current criteria at the time of presentation. Classification of antineutrophil cytoplasm autoantibody-associated small vessel vasculitis based on antineutrophil cytoplasm autoantibody specificity has been complicated by the finding that different ethnic groups may have very different clinical features relative to antigen specificity; for example, most patients with Wegener's granulomatosis in China have myeloperoxidase-antineutrophil cytoplasm autoantibodies rather than proteinase 3-antineutrophil cytoplasm autoantibodies. SUMMARY: Within the past year, new classification systems for primary vasculitis have been proposed, new classification criteria have been developed, and the appropriateness of a longstanding eponym has been challenged.

The challenge of managing patients with polyarteritis nodosa.

PURPOSE OF REVIEW: In this short review we focus on the problems faced by clinicians caused by the changing definitions of polyarteritis nodosa. RECENT FINDINGS: The term polyarteritis nodosa has been used for more than 100 years as a diagnostic term for patients with systemic vasculitis; however, specific vasculitides have been singled out like branches being chopped off a tree. Now, so little is left of the trunk of that tree that it is questionable to what extent we can trust older literature with respect to clinical features, natural history and response to treatment. Many authors of case reports, as well as authors of reviews and book chapters, claim they adhere to the Chapel Hill Consensus Conference definition of polyarteritis nodosa, yet still cite almost exclusively studies using older definitions without highlighting this dilemma. In the past year, two proposals affecting classification have been published: one stating that cutaneous polyarteritis nodosa and hepatitis-associated polyarteritis nodosa are diseases distinct from classical polyarteritis nodosa, and one providing an algorithm to separate microscopic polyangiitis from classical polyarteritis nodosa. SUMMARY: There is hope that a wide acceptance of the new classification principles will lead to a more uniform way to diagnose classical polyarteritis nodosa, which will facilitate clinical studies and eventually improve management.

Rokitansky and his first description of polyarteritis nodosa.

Karl Rokitansky, Professor of Pathology at the University of Vienna described polyarteritis nodosa in 1852 in a 23 year old man with a 5-day history of fever and diarrhoea.

Polyarteritis nodosa.

Polyarteritis nodosa (PAN) is regarded rightly as the grandfather of the vasculitides. In this Grand Rounds, the case of a 30-year-old man with a 12-year illness is described. The patient presented with daily fevers, tachycardia, and cutaneous ulcers on his distal extremities. He eventually developed mononeuritis multiplex. Because of the striking pattern of his fevers, he was diagnosed for many years as having adult-onset Still disease. Following the addition of daily cyclophosphamide to his long-standing regimen of prednisone, the patient's disease entered remission for the first time in more than a decade. He was ultimately able to discontinue all of his immunosuppressive medications. The case is discussed in the context of the first patient ever described with PAN, the classic report of Kussmaul and Maier.

Historical perspective of vasculitis: polyarteritis nodosa and microscopic polyangiitis.

The original and early case reports of vasculitis provide a historical context and foundation for understanding current concepts of these diseases. These early case reports are valuable as reference points for the current efforts in diagnosing, treating, and classifying vasculitis. In addition, they emphasize the importance of careful clinical observation in these efforts and the essential nature of medical science. Polyarteritis nodosa was the first noninfectious vasculitis to be described and studied in detail. Research on this group of vasculitides has been the cornerstone for understanding the pathophysiology of other forms of idiopathic vasculitis. Historically, most forms of vasculitis described subsequently have been characterized and classified on the basis of features similar to or distinct from polyarteritis.

Polyarteritis nodosa.

Polyarteritis nodosa is one of the earliest forms of systemic vasculitis described. It is characterized by segmental necrotizing arterial lesions affecting medium-sized and small-sized arteries. Active necrotizing lesions are frequently associated with chronic reparative lesions. Current convention would exclude patients with necrotizing glomerular lesions from this category. An aneurysm may form when the arterial wall is sufficiently weakened by the necrotizing process. Any organ may be affected; however, pulmonary involvement is very uncommon. Significant potential exists for end organ injury resulting from ischemia, infarcts, and hemorrhage. A variety of other primary systemic vasculitidies, secondary forms, such as connective tissue disease associated, and organ limited forms, have been identified that may exhibit similar arterial lesions including aneurysm formation. Establishing a definitive diagnosis of polyarteritis nodosa is therefore challenging, and mandates dialogue between the pathologist and the clinician primed with pertinent clinical and laboratory data. Fortunately, with early diagnosis and aggressive treatment with cytotoxic agents, the prognosis of polyarteritis nodosa has substantially improved.