Caroline OS Savage, MD, PhD, FRCP
presence of fibrinoid necrosis and inflammation of blood vessel walls.
Spectrum of Vasculitis
There is no classification of vasculitis that is accepted without dispute.
Vasculitic disorders may be divided into primary and secondary, distinguishing
vasculitides in which the vasculitic process is the main focus of tissue injury
(e.g. giant cell arteritis, microscopic polyangiitis) from those deemed to be
associated with another underlying disease(e.g. systemic lupus
erythematosus, rheumatoid arthritis, malignancy). Another division is between
vasculitides associated with the presence of antineutrophil cytoplasm
antibodies(ANCA) and those in which ANCA are seldom detected. The three
ANCA-associated forms of vasculitis are Wegener`s granulomatosis,
microscopic polyangiitis and Churg- Strauss syndrome. Closer to a system of
classification is subdivision of the primary systemic vasculitides according to
the predominant size and type of the vessel affected and/or whether there
are associated granulomata.
Despite the term "systemic", vasculitis may be localized to a single organ
and may or may not become systemic. This is illustrated by so-
called "idiopathic rapidly progressive glomerulonephritis", which is now
recognized as a form of microscopic polyangiitis limited to the kidney. A limited
form of Wegener`s granulomatosis also occurs, affecting the head and neck;
this is usually granulomatous rather than vasculitic initially, but may develop
into a systemic vasculitic disease.
Definitions
The primary systemic vasculitides are the most difficult to define because of
the lack of known aetiological factors in most cases and the overlap of clinical
features between syndromes. Nevertheless, several syndromes have been
recognized. In 1994, an international consensus conference attempted to
define the main syndromes to facilitate international understanding and
awareness. No other system has yet superseded the 1994 proposals.
Relationship to Age
Vasculitis may occur at any age, but the clinical syndromes with which it is
associated show age-specific differences. Kawasaki disease is seen only in
children; Henoch-Schonlein purpura occurs mainly in children, but can present
in adults; microscopic polyangiitis and Wegener`s granulomatosis generally
present in adults; and giant cell arteritis is predominantly a disease of the
elderly.
Importance of Diagnostic
Precision
Distinguishing subsets of vasculitis is justified because the aetiological
factors or pathogenic mechanisms are likely to differ, the requirements for
therapy differ, and prognosis varies between different syndromes. Precision is
also necessary for therapeutic, epidemiological and national or international
studies. Vasculitides are medical emergencies. Organ survival can be
threatened if a diagnosis is not made quickly and accurately, enabling
implementation of appropriate therapy. Blindness may occur in an elderly
patient in whom giant cell arteritis is missed, cardiac infarction may occur in a
child with Kawasaki disease, and renal failure and lifethreatening lung
haemorrhage may develop in an adult with microscopic polyangiitis or
Wegener`s granulomatosis. If in doubt about a diagnosis, consult an
appropriate specialist.
■ 기사 요지
본지 자매지 `MEDICAL PROGRESS` 5월호에 게재된 글로, 전신성 혈관염(맥관염)에 대
한 개요다.
전신성 혈관염은 섬유소양괴사(fibrinoid necrosis)와 혈관벽 염증에 의해 특징 지어지는
종합적 혈관장애를 의미한다. 전신성 혈관염은 병변의 크기와 영향을 받는 혈관위치에 따라
대혈관염·중혈관염·소혈관염으로 구분할 수 있다.
혈관염은 모든 연령대에서 발생하지만, 이로 인해 발생하는 임상증후군은 연령과 특별한 관
계가 있다. 가와사끼병은 소아에게서만 발견되며, 헤노흐-쇤라인 자반증은 주로 어린이에게
서 발생하나 성인에게서도 나타난다. 현미경적다발성맥관염(microscopic polyangiitis)과
베게너육아종증(Wegener`s granulomatosis)은 일반적으로 성인에게서 발견된다. 한편,
거대세포동맥염(giant cell arteritis)은 노령층에서 주로 나타나는 질환이다.
정리·이상돈 기자 sdlee@kimsonline.co.kr