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A Commentary
A Commentary . 1101
A Commentary on Pulmonary Granulomatous (Giant Cell) Vasculitides
J. T. Lie Sacramento, u.S.A. Pulmonary vasculitis has many causes and a variety of clinical and morphologic expressions (Table)I,2. Among the granulomatous (giant cell) variety of pulmonary vaculitis, the most common and best known types belong to the group of pulmonary angiitis and granulomatosis (PAG), initially classified by Liebow 3 . PAG consists of Wegener's granulomatosis (classic and limited types), Churg-Strauss syndrome (allergic granulomatosis and angiitis), necrotizing sarcoid granulomatosis, and angiitis associated bronchocentric granulomatosis, all of which affect predominantly, if not exclusively, the small parenchymal arteries and veins of the lung. The only other type of small-vessel granulomatous pulmonary vasculitis is the rare instance of lung involvement in disseminated visceral giant cell angiitis 4 • Large-vessel granulomatous or giant cell pulmonary angiitis occurs quite commonly ( ± 40%) in Takayasu arteritis 5 ,6 and only infrequently in giant cell (tempor-
Table Classification of pulmonary angiitis and granulomatosis Vasculitis of know cause and confined to the lungs
• Infective vasculitis (bacterial, fungal, parasitic, viral) • Reactive vasculitis to embolic material (cotton, gauze, talc) • Primary pulmonary hypertension (plexogenic arteriopathy) • Secondary pulmonary hypertension (recurrent thromboembolism) • Pulmonary veno-occlusive disease • Pulmonary hypertension associated with cirrhosis or anorectic drug
al) arteritis 7 - 10 • They can be distinguished by the age and gender of the patients, and the presence or absence of temporal arteritis clinically. Both Takayasu arteritis and temporal arteritis with lung involvement may also occasionally affect the smaller pulmonary arteries and veins 8 - 10 , and not all reported cases of pulmonary involvement in giant cell arteritis showed vasculitis; some merely had nodular or interstitial lung disease 7,11 Isolated pulmonary giant cell vasculitis was first described by Wagenaar et al in 1981 12 and, since then, at least three other cases have been added to the literature 13-15 before the present case report by Masuda et aP6. Although Masuda et aP6 emphasize theirs was the only autopsy case, there is not true 14 ; all other cases have satisfactorily excluded extrapulmonary giant cell arteritis 13-15. It should also be pointed out that the isolated pulmonary giant cell vasculitis in the patient described by Masuda et aP6 was an incidental autopsy finding; in all four other cases reported I2 - 15 , the patients were symptomatic: three required an operation for the relief of symptoms 12, 13,]5 while one died before surgical intervention was attempted 14 • To summarize, isolated pulmonary giant cell vasculitis, although rare, is not a benign disease. Clinically and angiographically, it can be distinguished from pulmonary angiitis and granulomatosis and from Takayasu arteritis and giant cell (temporal) arteritis with pulmonary involvement. With the limited clinical experience currently available for this rare form of pulmonary vasculitis, there has been no report of recurrence in those cases where surgical resection was feasible and carried out l2 , 13, 15.
Vasculitis of unknown cause with lung as primary target organ (pulmoary angiitis and granulomatosis)
• Wegener's granulomatosis (classic and limited forms) • Allergic angiitis and granulomatosis (Churg-Strauss syndrome) • Necrotizing sarcoid granulomatosis • Bronchocentric granulomatosis Systemic vasculitis that may involve the lungs
• • • • • • • • • •
Polyarteritis nodosa Rheumatoid arthritis Systemic lupus erythematosus Sceroderma (systemic sclerosis) Dermatopolymyositis Mixed connective tissue disease Hypersensitivity vasculitis Behcet syndrome/Hughes-Stovin syndrome Takayasu arteritis and giant cell (temporal) arteritis Disseminated and isolated giant cell angiitis
References 1 Lie JT (1977) Nosology of pulmonary vasculitides. Mayo Clin Proc 52: 520-522 2 Lie JT (1989) Classification of pulmonary angiitis and granulomatosis: Histopathologic perspectives. Semin Respir Med 10: 111-121 3 Liebow AA (1973) The J Burns Ambersons Lecture: Pulmonary angiitis and granulomatosis. Am Rev Respir Dis 108:
1-18 4 Lie JT (1978) Disseminated visceral giant cell arteritis: Histopathologic description and differential diagnosis from other" granulomatous vasculitides. Am J Clin Pathol 69:
299-305 5 Lupi EH, Sanchez GT, Horitz S, Gutierrez E (1975) Pulmonary artery involvement in Takayasu's arteritis. Chest 67:
69-74
1102 . ]. T. Lie 6 Shar~a S, Kamalakar T, Rajani M, Talwar KK, Shivastava S (1990) The incidence and patterns of pulmonary artery involvement in Takayasu's arteritis. Clin Radiol42: 177 -181 7 Karam GH, Fulmer JD (1982) Giant cell arteritis presenting as interstitial lung disease. Chest 82: 781-784 8 Bradley JD, Pinals RS, Blumenfeld HB, Poston WM (1984) Giant cell arteritis with pulmonary nodules. Am J Med 77: 135-140 9 Ladanyi M, Fraser RS (1987) Pulmonary involvement in giant cell arteritis. Arch Pathol Lab Med 111: 1178 -1180 10 Doyle L, McWilliam L, HasletonPS (1988) Giant cell arteritis with pulmonary involvement. Br J Dis Chest 82: 8892 11 Kramer MR, Melzer E, Nesher G, Sonnenblick M (1987) Pulmonary manifestations of temporal arteritis. Eur J Respir Dis 71: 430-433
12 Wagenaar sse, Westermann CJJ, Corrin B (1981) Giant cell arteritis limited to large elastic pulmonary arteries. Thorax 36: 876-877 13 Wagenaar SS, van den Bosch JMM, Westermann CJJ, Boseman HG, Lie JT (1986) Isolated granulomatous giant cell vasculitis of the pulmonary elastic arteries. Arch Pathol Lab Med 110: 962-964 14 Glover MU, Muniz J, Bessone L, Carta M, Casellas J, Maniscalco BS (1987) Pulmonary artery obstruction due to giant cell arteritis. Ches 91: 924- 925 15 Okubo S, Kunieda T, Ando M, Nakajima N, Yutani C (1988) Idiopathic isolated pulmonary arteritis with chronic cor pulmonale. Chest 94: 665 -666 16 Masuda S, Ishii T, Asuwa N, Ishikawa Y, Kiguchi H (1994) Isolated pulmonary giant cell vasculitis. Path Res Pract 190:
Professor Dr. med. ]. T. Lie, Department of Pathology, UC Davis Medical Center, PAT-I005, 2315 Stockton Bouleward, Sacramento, California 95817, USA
A Commentary on Pulmonary Granulomatous (Giant Cell) Vasculitides
J. T. Lie Sacramento, u.S.A. Pulmonary vasculitis has many causes and a variety of clinical and morphologic expressions (Table)I,2. Among the granulomatous (giant cell) variety of pulmonary vaculitis, the most common and best known types belong to the group of pulmonary angiitis and granulomatosis (PAG), initially classified by Liebow 3 . PAG consists of Wegener's granulomatosis (classic and limited types), Churg-Strauss syndrome (allergic granulomatosis and angiitis), necrotizing sarcoid granulomatosis, and angiitis associated bronchocentric granulomatosis, all of which affect predominantly, if not exclusively, the small parenchymal arteries and veins of the lung. The only other type of small-vessel granulomatous pulmonary vasculitis is the rare instance of lung involvement in disseminated visceral giant cell angiitis 4 • Large-vessel granulomatous or giant cell pulmonary angiitis occurs quite commonly ( ± 40%) in Takayasu arteritis 5 ,6 and only infrequently in giant cell (tempor-
Table Classification of pulmonary angiitis and granulomatosis Vasculitis of know cause and confined to the lungs
• Infective vasculitis (bacterial, fungal, parasitic, viral) • Reactive vasculitis to embolic material (cotton, gauze, talc) • Primary pulmonary hypertension (plexogenic arteriopathy) • Secondary pulmonary hypertension (recurrent thromboembolism) • Pulmonary veno-occlusive disease • Pulmonary hypertension associated with cirrhosis or anorectic drug
al) arteritis 7 - 10 • They can be distinguished by the age and gender of the patients, and the presence or absence of temporal arteritis clinically. Both Takayasu arteritis and temporal arteritis with lung involvement may also occasionally affect the smaller pulmonary arteries and veins 8 - 10 , and not all reported cases of pulmonary involvement in giant cell arteritis showed vasculitis; some merely had nodular or interstitial lung disease 7,11 Isolated pulmonary giant cell vasculitis was first described by Wagenaar et al in 1981 12 and, since then, at least three other cases have been added to the literature 13-15 before the present case report by Masuda et aP6. Although Masuda et aP6 emphasize theirs was the only autopsy case, there is not true 14 ; all other cases have satisfactorily excluded extrapulmonary giant cell arteritis 13-15. It should also be pointed out that the isolated pulmonary giant cell vasculitis in the patient described by Masuda et aP6 was an incidental autopsy finding; in all four other cases reported I2 - 15 , the patients were symptomatic: three required an operation for the relief of symptoms 12, 13,]5 while one died before surgical intervention was attempted 14 • To summarize, isolated pulmonary giant cell vasculitis, although rare, is not a benign disease. Clinically and angiographically, it can be distinguished from pulmonary angiitis and granulomatosis and from Takayasu arteritis and giant cell (temporal) arteritis with pulmonary involvement. With the limited clinical experience currently available for this rare form of pulmonary vasculitis, there has been no report of recurrence in those cases where surgical resection was feasible and carried out l2 , 13, 15.
Vasculitis of unknown cause with lung as primary target organ (pulmoary angiitis and granulomatosis)
• Wegener's granulomatosis (classic and limited forms) • Allergic angiitis and granulomatosis (Churg-Strauss syndrome) • Necrotizing sarcoid granulomatosis • Bronchocentric granulomatosis Systemic vasculitis that may involve the lungs
• • • • • • • • • •
Polyarteritis nodosa Rheumatoid arthritis Systemic lupus erythematosus Sceroderma (systemic sclerosis) Dermatopolymyositis Mixed connective tissue disease Hypersensitivity vasculitis Behcet syndrome/Hughes-Stovin syndrome Takayasu arteritis and giant cell (temporal) arteritis Disseminated and isolated giant cell angiitis
References 1 Lie JT (1977) Nosology of pulmonary vasculitides. Mayo Clin Proc 52: 520-522 2 Lie JT (1989) Classification of pulmonary angiitis and granulomatosis: Histopathologic perspectives. Semin Respir Med 10: 111-121 3 Liebow AA (1973) The J Burns Ambersons Lecture: Pulmonary angiitis and granulomatosis. Am Rev Respir Dis 108:
1-18 4 Lie JT (1978) Disseminated visceral giant cell arteritis: Histopathologic description and differential diagnosis from other" granulomatous vasculitides. Am J Clin Pathol 69:
299-305 5 Lupi EH, Sanchez GT, Horitz S, Gutierrez E (1975) Pulmonary artery involvement in Takayasu's arteritis. Chest 67:
69-74
1102 . ]. T. Lie 6 Shar~a S, Kamalakar T, Rajani M, Talwar KK, Shivastava S (1990) The incidence and patterns of pulmonary artery involvement in Takayasu's arteritis. Clin Radiol42: 177 -181 7 Karam GH, Fulmer JD (1982) Giant cell arteritis presenting as interstitial lung disease. Chest 82: 781-784 8 Bradley JD, Pinals RS, Blumenfeld HB, Poston WM (1984) Giant cell arteritis with pulmonary nodules. Am J Med 77: 135-140 9 Ladanyi M, Fraser RS (1987) Pulmonary involvement in giant cell arteritis. Arch Pathol Lab Med 111: 1178 -1180 10 Doyle L, McWilliam L, HasletonPS (1988) Giant cell arteritis with pulmonary involvement. Br J Dis Chest 82: 8892 11 Kramer MR, Melzer E, Nesher G, Sonnenblick M (1987) Pulmonary manifestations of temporal arteritis. Eur J Respir Dis 71: 430-433
12 Wagenaar sse, Westermann CJJ, Corrin B (1981) Giant cell arteritis limited to large elastic pulmonary arteries. Thorax 36: 876-877 13 Wagenaar SS, van den Bosch JMM, Westermann CJJ, Boseman HG, Lie JT (1986) Isolated granulomatous giant cell vasculitis of the pulmonary elastic arteries. Arch Pathol Lab Med 110: 962-964 14 Glover MU, Muniz J, Bessone L, Carta M, Casellas J, Maniscalco BS (1987) Pulmonary artery obstruction due to giant cell arteritis. Ches 91: 924- 925 15 Okubo S, Kunieda T, Ando M, Nakajima N, Yutani C (1988) Idiopathic isolated pulmonary arteritis with chronic cor pulmonale. Chest 94: 665 -666 16 Masuda S, Ishii T, Asuwa N, Ishikawa Y, Kiguchi H (1994) Isolated pulmonary giant cell vasculitis. Path Res Pract 190:
Professor Dr. med. ]. T. Lie, Department of Pathology, UC Davis Medical Center, PAT-I005, 2315 Stockton Bouleward, Sacramento, California 95817, USA