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The Pulmonary Vasculitides
todav's practice or cardiOPulmonary mediCine The Pulmonary Vasculitides Jack D. Fulmer, M.D., F.C.C.P.; 0 and H. Benfer Kaltreider, M.D., F.C.C.P.t
v
asculitis is defined as inflammation of blood vessel walls. 1•2 Many of the vasculitides exhibit destruction of vessel walls so that the process is a necrotizing one. In some of the vasculitides, the inflammatory cells are primarily neutrophils or eosinophils; in others, the cellular response is mainly lymphocytic or granulomatous. 1•2 A few of the vasculitides are characterized by infiltration of vessel walls with cells that are unique to the underlying disease process such as in certain lymphoproliferative disorders. The pulmonary vasculitides consl'itute a heterogeneous group of disorders. Many of these occur in association with a disseminated vasculitis, while others are confined mainly to the lung. This review will present an overview of the pulmonary vasculitides. A classification will be presented and current concepts of the pathogenesis of these disorders will be discussed. Finally, an approach to the diagnosis and treatment of these disorders will be outlined. CLASSIFICATION
Classification of the vasculitides is at present arbitrary, but necessary, in order to approach these diseases in an orderly fashion. Several classifications have been proposed in the past. 1•2 The present classification separates the pulmonary vasculitides into several groups: those in which the lung is the major organ involved, and those in which lung is involved in association with a disseminated vasculitic process (Table 1). An additional group of diseases consists of those disorders in which pulmonary vasculitis is an integral part of the spectrum of pathology of the underlying disease (Table 2). Some of the From the Veterans Administration Medical Center, Birmingham, Alabama and San Francisco, California; •the Pulmo~ Division, Del_)artment of Medicine, University of Alabama in Birmingham; and tthe Cardiovascular Research Institute, and Department of Medicine, University of California, San Francisco. Supported in part by funds from the Research Service of the Veterans Administration (Birmingham and San Francisco). Reprint requests: Dr. Fulmer, Pulmonary Division/Medicine, University of AlabatrUJ in Birmingham, University Station Birmingham 35294 '
pulmonary vasculitides constitute unique clinical syndromes; others must be defined by specific clinicopathologic features or by associated disease processes. 1 The main vasculitides in which the lung is the major organ involved include the Churg-Strauss syndrome, Wegener's granulomatosis, and lymphomatoid granulomatosis. 1 Often classified with the latter disease, lymphocytic angiitis and granulomatosis is sometimes regarded as a separate and more benign disease. 1•3 The term overlap vasculitis has been coined recently to classify these disorders which have features of several vasculitic syndromes Table !-Pulmonary J'GKulitid~
Vasculitides in which lung is the major organ involved: Wegener's granulomatoEis Lymphomatoid granulomat()!;is Lymphocytic angiitis and granulomatosis Churg-Stralll's syndrome Overlap vasculitis Necrotizing sarcoid granulomatosis Vasculitides in which lung may be involved: Henoch-Schoenlein syndrome Disseminated leukocytoclastic vasculitis Cryoglobulinemia DiEseminated giant cell arteritis Behcet's disease Takayasu's disease Polyarteritis nodose. Table ~Pulmonary J'GKulitide.
Diseases in which pulmonary vasculitis may be part of the spectrum of pathology : Collagen-vascular disorders Rheumatoid arthritis Systemic lupus erythematosus Progressive systemic sclerosis Eosinophilic pneumonias Sarcoidosis Immunoblastic lymphadenopathy Organic dust diseases (hypersensitivity pneumonitides) Bronchocentric granulomatosis Ulcerative colitis Ankylosing spondylitis Hughes-Stovin syndrome
CHEST I 82 I 5 I NOVEMBER, 1982
815
v
asculitis is defined as inflammation of blood vessel walls. 1•2 Many of the vasculitides exhibit destruction of vessel walls so that the process is a necrotizing one. In some of the vasculitides, the inflammatory cells are primarily neutrophils or eosinophils; in others, the cellular response is mainly lymphocytic or granulomatous. 1•2 A few of the vasculitides are characterized by infiltration of vessel walls with cells that are unique to the underlying disease process such as in certain lymphoproliferative disorders. The pulmonary vasculitides consl'itute a heterogeneous group of disorders. Many of these occur in association with a disseminated vasculitis, while others are confined mainly to the lung. This review will present an overview of the pulmonary vasculitides. A classification will be presented and current concepts of the pathogenesis of these disorders will be discussed. Finally, an approach to the diagnosis and treatment of these disorders will be outlined. CLASSIFICATION
Classification of the vasculitides is at present arbitrary, but necessary, in order to approach these diseases in an orderly fashion. Several classifications have been proposed in the past. 1•2 The present classification separates the pulmonary vasculitides into several groups: those in which the lung is the major organ involved, and those in which lung is involved in association with a disseminated vasculitic process (Table 1). An additional group of diseases consists of those disorders in which pulmonary vasculitis is an integral part of the spectrum of pathology of the underlying disease (Table 2). Some of the From the Veterans Administration Medical Center, Birmingham, Alabama and San Francisco, California; •the Pulmo~ Division, Del_)artment of Medicine, University of Alabama in Birmingham; and tthe Cardiovascular Research Institute, and Department of Medicine, University of California, San Francisco. Supported in part by funds from the Research Service of the Veterans Administration (Birmingham and San Francisco). Reprint requests: Dr. Fulmer, Pulmonary Division/Medicine, University of AlabatrUJ in Birmingham, University Station Birmingham 35294 '
pulmonary vasculitides constitute unique clinical syndromes; others must be defined by specific clinicopathologic features or by associated disease processes. 1 The main vasculitides in which the lung is the major organ involved include the Churg-Strauss syndrome, Wegener's granulomatosis, and lymphomatoid granulomatosis. 1 Often classified with the latter disease, lymphocytic angiitis and granulomatosis is sometimes regarded as a separate and more benign disease. 1•3 The term overlap vasculitis has been coined recently to classify these disorders which have features of several vasculitic syndromes Table !-Pulmonary J'GKulitid~
Vasculitides in which lung is the major organ involved: Wegener's granulomatoEis Lymphomatoid granulomat()!;is Lymphocytic angiitis and granulomatosis Churg-Stralll's syndrome Overlap vasculitis Necrotizing sarcoid granulomatosis Vasculitides in which lung may be involved: Henoch-Schoenlein syndrome Disseminated leukocytoclastic vasculitis Cryoglobulinemia DiEseminated giant cell arteritis Behcet's disease Takayasu's disease Polyarteritis nodose. Table ~Pulmonary J'GKulitide.
Diseases in which pulmonary vasculitis may be part of the spectrum of pathology : Collagen-vascular disorders Rheumatoid arthritis Systemic lupus erythematosus Progressive systemic sclerosis Eosinophilic pneumonias Sarcoidosis Immunoblastic lymphadenopathy Organic dust diseases (hypersensitivity pneumonitides) Bronchocentric granulomatosis Ulcerative colitis Ankylosing spondylitis Hughes-Stovin syndrome
CHEST I 82 I 5 I NOVEMBER, 1982
815