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Granulomatous Vasculitides of the Lung: A Clinicopathologic Approach to Diagnosis and Treatment

Granulomatous Vasculitides of the Lung: A Clinicopathologic Approach to Diagnosis and Treatment and histopathologic features of granulomatous vasculitides of the lung overlap considerably. Although certain radiologic appearances faLINICAL clinically and histologically mimics. Radiographs von a particular Because effective diagnosis, therapy these depends findings on an exact also vary. diag- nosis, an integrated clinical and is needed to accurately diagnose promptly. There are two groups vasculitides: true and lymphoproliferative. histologic approach these processes of gnanulomatous prototypical true Wegener granulomatosis is the granulomatous vasculitis. A necrotizing, granulomatous inflammation involves the vessels of multiple organs, predominantly those of the lungs, upper respiratory disorder tract, have and kidneys. respiratory All patients disease, which with this usually pre- show pulmonary infiltrates, frequently in a nodular or segmental pattern, and occasionally hilar lymphadenopathy. This is in contrast to radiographs of sancoidosis, which usually show bilateral hilar lymphadenopathy, with or without interstitial infiltrates. Bronchocentnic granulomatosis can also mimic true granulomatous vasculitides. It is not a true vasculitis because angiopathy is minor and secondary to necnotizing, granulomatous destruction of airways. Radiographs usually reveal segmental or lobar milltrates, appearing as discrete, solitary masses. cedes renal disease. The pulmonary cellular infiltrate is mostly mononuclear and consists of many histiocytes and giant cells; varying numbers of plasma cells, neutrophils, and eosinophils; and lymphocytes only rarely. Evidence of glomerulonephritis is seen in most patients; ocular disease and otitis media are also common. The most common radiographic findings are multiple nodular infiltrates at the lung bases that change in size, may migrate, and tend toward cavitation. The cause of Wegener granulomatosis is unknown: Some investigators suspect immune Lymphomatoid granulomatosis (LYG) is the prototype of the second major group, lymphoproliferative granulomatous vasculitides. Clinical features Indude pulmonary infiltrates, skin lesions, and central nervous system dysfunction; although the kidney may be involved, glomerulonephritis is never found. Granulomas, if present, occur in much smallen numbers than in the true vasculitides. Pulmonary lesions produce an extensive coagulative necrosis of the panenchyma sels, which atypical that surrounds involved blood yesnot inflamed but are infiltrated with mononuclear cells. The cells consist of small are rounded large complexes may contribute to pathogenesis; others question this. Prognosis previously was grim, but the accepted, most recent treatment, with cyclophosphamide and prednisone, has resulted in increased remission and survival rates. lymphocytes, stimulated lymphocytes, and atypical reticular elements. Plasma cells, atypical plasmacytoid lymphocytes, and mitoses are frequent; eosinophils and neutrophils are uncommon. Radiographic findings are not diagnostic, but the most common include multiple nodules and masses that may cavitate. The cause of LYG is unknown, but A second type, allergic granulomatosis and angiitis (also known as Churg-Strauss syndrome), is characterized by bronchial asthma; peripheral eosino- some believe viral philia; trates; diffuse, and patchy, on nodular vasculitis. pulmonary infilsites suggest that the nosis has been extremely poor. Cyclophosphamide and pnednisone have been used with some success, infections may play a role; disease is a T cell lymphoma. others Prog- systemic Extrapulmonary include the heart, kidneys, mesentery, skin, and muscle, but significant impairment of renal function is uncommon. Pulmonary lesions consist of extensive vasculitis and necrosis, with intense infiltration of eosinophils in blood vessel walls and perivascular tissue. Granulomas are often found. The disease course may range from an indolent process to a fulminating fatal vasculitis. Corticosteroids help im- but in some patients so treated, LYG has evolved into lymphoma. Several investigators have recommended centric chemotherapy lymphoproliferative protocols. LYG must be distinguished from processes: two other benign angiolym- prove survival; immunosuppressive helpful in patients with extrapulmonary ment. therapy may involvegranulomatosis, infiltrates, and be phocytic angiitis and granulomatosis (BLAG) and large cell lymphoma with angioinvasion. BLAG is almost always limited to the lung and produces less pronounced symptoms than LYG. Lymphoma has foci of large monomorphous atypical lymphoid cells not found in LYG. These diseases most likely consti- A third is characterized type, necrotizing by nodular sarcoid pulmonary absence course. osis and and of extrapulmonary Histologic features Wegener confluent granulomatosis vasculitis, resemble and those a benign of sarcoidexten- include sive tis, parenchymal necrosis, panenchymal granulomatous granulomas vasculicom- tute a spectrum of progressively proliferative processes, since into LYG, and LYG into large cause BLAG is more successfully and LYG is more successfully ma, early, aggressive therapy obligatory. U SUSAN POOLE HARMON abnormal lymphoBLAG can transform cell lymphoma. Betreated than LYG treated than lymphofor these disorders is posed of epitheloid histiocytes extensiveness of coagulative distinguishes this disease from and giant on fibrinoid sarcoidosis, cells. The necrosis which it RSNA Publications #{149} Radiology November http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Radiology Radiological Society of North America, Inc.

Granulomatous Vasculitides of the Lung: A Clinicopathologic Approach to Diagnosis and Treatment

SUSAN POOLE HARMON
Radiology , Volume 173: 450 – Nov 1, 1989
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Publisher
Radiological Society of North America, Inc.
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Copyright © November 1989 by Radiological Society of North America
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1527-1315
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0033-8419
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Abstract

and histopathologic features of granulomatous vasculitides of the lung overlap considerably. Although certain radiologic appearances faLINICAL clinically and histologically mimics. Radiographs von a particular Because effective diagnosis, therapy these depends findings on an exact also vary. diag- nosis, an integrated clinical and is needed to accurately diagnose promptly. There are two groups vasculitides: true and lymphoproliferative. histologic approach these processes of gnanulomatous prototypical true Wegener granulomatosis is the granulomatous vasculitis. A necrotizing, granulomatous inflammation involves the vessels of multiple organs, predominantly those of the lungs, upper respiratory disorder tract, have and kidneys. respiratory All patients disease, which with this usually pre- show pulmonary infiltrates, frequently in a nodular or segmental pattern, and occasionally hilar lymphadenopathy. This is in contrast to radiographs of sancoidosis, which usually show bilateral hilar lymphadenopathy, with or without interstitial infiltrates. Bronchocentnic granulomatosis can also mimic true granulomatous vasculitides. It is not a true vasculitis because angiopathy is minor and secondary to necnotizing, granulomatous destruction of airways. Radiographs usually reveal segmental or lobar milltrates, appearing as discrete, solitary masses. cedes renal disease. The pulmonary cellular infiltrate is mostly mononuclear and consists of many histiocytes and giant cells; varying numbers of plasma cells, neutrophils, and eosinophils; and lymphocytes only rarely. Evidence of glomerulonephritis is seen in most patients; ocular disease and otitis media are also common. The most common radiographic findings are multiple nodular infiltrates at the lung bases that change in size, may migrate, and tend toward cavitation. The cause of Wegener granulomatosis is unknown: Some investigators suspect immune Lymphomatoid granulomatosis (LYG) is the prototype of the second major group, lymphoproliferative granulomatous vasculitides. Clinical features Indude pulmonary infiltrates, skin lesions, and central nervous system dysfunction; although the kidney may be involved, glomerulonephritis is never found. Granulomas, if present, occur in much smallen numbers than in the true vasculitides. Pulmonary lesions produce an extensive coagulative necrosis of the panenchyma sels, which atypical that surrounds involved blood yesnot inflamed but are infiltrated with mononuclear cells. The cells consist of small are rounded large complexes may contribute to pathogenesis; others question this. Prognosis previously was grim, but the accepted, most recent treatment, with cyclophosphamide and prednisone, has resulted in increased remission and survival rates. lymphocytes, stimulated lymphocytes, and atypical reticular elements. Plasma cells, atypical plasmacytoid lymphocytes, and mitoses are frequent; eosinophils and neutrophils are uncommon. Radiographic findings are not diagnostic, but the most common include multiple nodules and masses that may cavitate. The cause of LYG is unknown, but A second type, allergic granulomatosis and angiitis (also known as Churg-Strauss syndrome), is characterized by bronchial asthma; peripheral eosino- some believe viral philia; trates; diffuse, and patchy, on nodular vasculitis. pulmonary infilsites suggest that the nosis has been extremely poor. Cyclophosphamide and pnednisone have been used with some success, infections may play a role; disease is a T cell lymphoma. others Prog- systemic Extrapulmonary include the heart, kidneys, mesentery, skin, and muscle, but significant impairment of renal function is uncommon. Pulmonary lesions consist of extensive vasculitis and necrosis, with intense infiltration of eosinophils in blood vessel walls and perivascular tissue. Granulomas are often found. The disease course may range from an indolent process to a fulminating fatal vasculitis. Corticosteroids help im- but in some patients so treated, LYG has evolved into lymphoma. Several investigators have recommended centric chemotherapy lymphoproliferative protocols. LYG must be distinguished from processes: two other benign angiolym- prove survival; immunosuppressive helpful in patients with extrapulmonary ment. therapy may involvegranulomatosis, infiltrates, and be phocytic angiitis and granulomatosis (BLAG) and large cell lymphoma with angioinvasion. BLAG is almost always limited to the lung and produces less pronounced symptoms than LYG. Lymphoma has foci of large monomorphous atypical lymphoid cells not found in LYG. These diseases most likely consti- A third is characterized type, necrotizing by nodular sarcoid pulmonary absence course. osis and and of extrapulmonary Histologic features Wegener confluent granulomatosis vasculitis, resemble and those a benign of sarcoidexten- include sive tis, parenchymal necrosis, panenchymal granulomatous granulomas vasculicom- tute a spectrum of progressively proliferative processes, since into LYG, and LYG into large cause BLAG is more successfully and LYG is more successfully ma, early, aggressive therapy obligatory. U SUSAN POOLE HARMON abnormal lymphoBLAG can transform cell lymphoma. Betreated than LYG treated than lymphofor these disorders is posed of epitheloid histiocytes extensiveness of coagulative distinguishes this disease from and giant on fibrinoid sarcoidosis, cells. The necrosis which it RSNA Publications #{149} Radiology November

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Published: Nov 1, 1989

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