Pulmonary Manifestations of the Churg-Strauss Syndrome
Pulmonary Manifestations of the Churg-Strauss Syndrome
Purpose of review: Pulmonary vascular involvement and the resulting clinical manifestations are prominent in several of the idiopathic systemic small vessel vasculitis syndromes. This group of syndromes, often referred to as the antineutrophil cytoplasmic antibody-associated vaculitis syndromes, is composed of the Churg-Strauss syndrome, Wegener's granulomatosis, microscopic polyangiitis, and several organ specific subsets of the latter. The former three syndromes form the basis of this review.
Recent findings: Because the presenting signs and symptoms are variable and because these are uncommon disorders, the clinical manifestations in different age groups, ethnic groups, sexes, stage of disease among others have been documented by way of a number of case series and case reports. The role of the antineutrophil cytoplasmic antibody in these disorders is being clarified. In diagnosis, the usefulness of the test depends on the sensitivity and specificity of testing in the population being studied. In pathogenesis, the antineutrophil cytoplasmic antibody may be causative or may merely be associated with the clinical manifestations.
Summary: These disorders, although distinctive, are often difficult to diagnose and commonly pose a perplexing clinical scenario for the clinician. A thorough history, physical examination, and a high index of suspicion is mandatory if these entities are to be recognized expeditiously.
Pulmonary vasculitis is a common finding in a number of uncommon disease entities. Pulmonary vessel involvement may be a significant part of a systemic vasculitis or may be the sole/predominant site of involvement. Pulmonary vasculitis is characterized pathologically by cellular inflammation, destruction of the blood vessel wall, and tissue necrosis. Clinically, pulmonary vasculitis is characterized by the size, type, and location of the affected vessels in association with the degree of inflammation, vessel destruction, and tissue necrosis. Because of the plethora of presenting signs and symptoms, an accurate and definitive diagnosis presents a challenge to even the expert clinician. A firm diagnosis relies on the recognition of a combination of radiologic, pathologic, laboratory, and clinical findings. Furthermore, a specific diagnosis may be elusive in that overlap syndromes are common.
Establishing a uniform classification scheme for the vasculitis syndromes has been likewise challenging. A commonly used classification scheme is listed in Table 1 . Here, the vasculitides are divided into primary idiopathic, primary immune-complex mediated, and secondary entities. The primary idiopathic category is further categorized by the size of vessel predominantly involved. In this paper, the small vessel, primary idiopathic vasculitis syndromes will be discussed. In these disorders, the vessels involved are predominantly the capillaries, venules, and arterioles. Medium-sized vessels (that is, the main visceral arteries such as the renal, hepatic, and coronary) may be involved on occasion. These entities are also known as the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes due to the frequent (though not uniform) association with these antibodies.
Purpose of review: Pulmonary vascular involvement and the resulting clinical manifestations are prominent in several of the idiopathic systemic small vessel vasculitis syndromes. This group of syndromes, often referred to as the antineutrophil cytoplasmic antibody-associated vaculitis syndromes, is composed of the Churg-Strauss syndrome, Wegener's granulomatosis, microscopic polyangiitis, and several organ specific subsets of the latter. The former three syndromes form the basis of this review.
Recent findings: Because the presenting signs and symptoms are variable and because these are uncommon disorders, the clinical manifestations in different age groups, ethnic groups, sexes, stage of disease among others have been documented by way of a number of case series and case reports. The role of the antineutrophil cytoplasmic antibody in these disorders is being clarified. In diagnosis, the usefulness of the test depends on the sensitivity and specificity of testing in the population being studied. In pathogenesis, the antineutrophil cytoplasmic antibody may be causative or may merely be associated with the clinical manifestations.
Summary: These disorders, although distinctive, are often difficult to diagnose and commonly pose a perplexing clinical scenario for the clinician. A thorough history, physical examination, and a high index of suspicion is mandatory if these entities are to be recognized expeditiously.
Pulmonary vasculitis is a common finding in a number of uncommon disease entities. Pulmonary vessel involvement may be a significant part of a systemic vasculitis or may be the sole/predominant site of involvement. Pulmonary vasculitis is characterized pathologically by cellular inflammation, destruction of the blood vessel wall, and tissue necrosis. Clinically, pulmonary vasculitis is characterized by the size, type, and location of the affected vessels in association with the degree of inflammation, vessel destruction, and tissue necrosis. Because of the plethora of presenting signs and symptoms, an accurate and definitive diagnosis presents a challenge to even the expert clinician. A firm diagnosis relies on the recognition of a combination of radiologic, pathologic, laboratory, and clinical findings. Furthermore, a specific diagnosis may be elusive in that overlap syndromes are common.
Establishing a uniform classification scheme for the vasculitis syndromes has been likewise challenging. A commonly used classification scheme is listed in Table 1 . Here, the vasculitides are divided into primary idiopathic, primary immune-complex mediated, and secondary entities. The primary idiopathic category is further categorized by the size of vessel predominantly involved. In this paper, the small vessel, primary idiopathic vasculitis syndromes will be discussed. In these disorders, the vessels involved are predominantly the capillaries, venules, and arterioles. Medium-sized vessels (that is, the main visceral arteries such as the renal, hepatic, and coronary) may be involved on occasion. These entities are also known as the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes due to the frequent (though not uniform) association with these antibodies.
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