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Radiation Therapy and Mechanical Dilation of Endobronchial Obstruction Secondary to Wegener's Granulomatosis
bottom). Follow-up in February 1979 revealed little change in the chest roentgenogram but marked improvement in the pulmonary function tests (Table 1 and Fig 2). The pleural eHusions have not recurred.
COMMENTS
Radiation Therapy and Mechanical Dilation of Endobronchial Obstruction Secondary to Wegener's Granulomatosis* Lanie E. Eagleton, M.D., F.C.C.P.;··
This case illustrates a very unusual pulmonary manifestation of sarcoidosis, with bilateral pleuritic pain as the initial symptom and the presence of a bloody pleural effusion. Although we have not obtained histological demonstration of the pleural involvement by the granulomatous process, several lines of evidence support the diagnosis of pleural sarcoidosis. First, we found no other cause of bloody pleural effusion, such as tuberculosis, viral infection, pulmonary embolism, or neoplasm, and biopsy of mediastinal lymph nodes showed lesions typically seen in sarcoidosis. Second, the pleural effusion, as seen by repeated chest roentgenograms, remained stable over a period of four months, whereas it disappeared within a few days under prednisone therapy and did not recur in the ensuing three months. Finally, the treatment resulted in marked improvement of parenchymal infiltrates and of pulmonary function tests; the evolution of the lung mechanical properties under steroid therapy was identical to the pattern observed in patients with well-established sarcoidosis." In conclusion, sarcoidosis should be included in the differential diagnosis of bloody pleural effusion.
Richa,d B. Boshe« MD.;t
Anthony Hawe, M.B., Ch.B., F.R.C.S.;t and Richa,d T. BiUnsky, M.D.t
A patient with Wegener's granulomatosis, which was otherwise weD controDed with steroids an' cyelophosphamide, bad persistent total obstruction of an in8amed left maiDstem bronchus. The inflammation resolved, and file hmg expanded following radiation therapy• However, Intermittent atelectasis and pneumonia occurred distal to a residual short stricture. Repeated dilation endoscopicaDy with Plummer boogies bas proven effective In maintaining good ventilation and preventiDI recurrence of the atelectasis and pneumonia in the left lung for 18 months. secondary to endobronchial obA telectasis However, struction in Wegener's can occur
granulomatosis.t-s
there is little precedent in the literature to aid in the management of this complication. We report the successful management of recurrent bronchial obstroction with the combination of cyclophosphamide, prednisone and local radiation to control inflammation and repeated mechanical bronchial dilation to maintain patency of the airway. CASE REPORT
1 Case Records of the Massachusetts General Hospital (Case 1-1979). N Engl J Med 300:28-37, 1979 2 Mayock RL, Bertrand P, Morrison CE: Manifestations of sarcoidosis: Analysis of 145 patients, with a review of nine series selected hom the literature. Am J Med 35:67-89, 1963 3 Beekman ]F, Zimmet SM, Chun BK, et a1: Spectrum of pleural involvement of sarcoidosis. Arch Intern Med 136: 323-330, 1976 4 Wilen SB, Rabinowitz IG, Ulreich S, et al: Pleural Involvement in sarcoidosis. Am J Moo57:200-209, 1974 5 Sharma PO: Sarcoidosis: Unusual pulmonary manifestations. Postgrad Moo: 61:67-73, 1977 6 Chusid EL, Siltzbach LE: Sarcoidosis of the pleura. Ann Intern Med 81:190-194, 1974 7 Berte SI, pfotenhauer MA: Massive pleural effusion in sarcoidosis. Am Rev R~ir Dis 80:251-254, 1962 8 Littner MR, Schachter EN, Putman CE, et al: The clinical assessment of roentgenographically atypical pulmonary sarcoidosis. Am J Med 62:361-367, 1977 9 De Troyer A, Yernault JC, Diercla P, et al: Lung and airway mechanics in early pulmonary sarcoidosis. Bull Europ Physiopathol Respir 14:299-310, 1978
CHEST, 76: 5, NOVEMBER, 1979
This 35-year-old woman had persistent rhinitis, sinusitis, otitis, and arthritis which was treated with gold and steroids until 1969 when she developed glomerulonephritis. Wegener's granulomatosis was diagnosed based upon several biopsies showing the following: "vasculitis with fibrinoid necrosis in skin and muscle; acute and chronic inflammation with multinucleated giant cells in nasal mucosa; and proliferative glomerulonephritis and vasculitis in the kidney. The creatinine clearance level improved from 50 to 110 L/24 hours following an increase in the steroid dose. In 1971, she had granulomatous tracheitis causing subglottic stenosis. This was repaired surgically in 1975. She had fleeting pulmonary nodules at that time. In May 1976, she had acute dyspnea and severe chest pain concomitant with atelectasis of the left lung. A tracheostomy, currently maintained with a Kistner plastic tracheostomy tube, was made to facilitate bronchoscopy. The bronchial mucosa was erythematous, edematous, and very friable. The left main bronchus was occluded with granulation tissue; the right main bronchus was 3 mm in diameter. She was treated with cyclophosphamide and prednisone. The lung re-ex·From the Departments of Medicine and Surgery, Southern Dlinois University School of Medicine, Springfield, IL. ••Assistant Professor.
tlnstructor.
tClinical Associate Professor. Reprint reqfJe8ta: Dr. Eagleton, SIU School of Medicine, PO BO% 3926, Springfield, IUinoiB 62708
WEGENER'S GRANULOMATOSIS •
FIGURE 1. (A) (left) Carina in January 1977. The right mainstem bronchus is approximately 6 rom in diameter. Mucus is plugging "pit-like" orifice of the left mainstem bronchus on the left. ( B) (center) Left mainstem bronchus after suctioning free mucus. Mucosa is normal and orifice is approximately 1 mm in diameter. (C) (right) Carina following mechanical dilation in March 1977. Orifice of left mainstem bronchus is now approximately 3 rom in diameter.
panded three weeks later, but collapsed again in August 1976 when the left main bronchus again occluded with inflamed tissue. Because of persistent atelectasis one month later, radiation (2,000 rads) was administered to the trachea and bronchi. Two weeks after completion of this treatment, the lung re-expanded. An additional 2,000 rads were given. In January 1977, there were brief recurrences of atelectasis secondary to mucous obstruction. The mucosa of the trachea and bronchi appeared normal. However, the left mainstem bronchus had a l-mm orifice which was obstructed with mucus (Fig 1) . As demonstrated by tomography, the stenotic segment of bronchus had a length of only 1 em. Poor ventilation and chest pain persisted. In March, the left lung was consolidated as a result of a pneumococcal pneumonia. One week later, dilation of the left mainstem bronchus was begun with Plummer esophageal bougies. During the ensuing two years, this mechanical dilation has been repeated °ten times at intervals of one to six months. There has been no recurrence of atelectasis or pneumonia in the left lung. Treatment with cyclophosphamide has been continued. Currently her creatinine clearance level and forced vital capacity (FVC) are within normal limits. The FEVt/FVC ratio is .62. She is a physically active housekeeper and mother.
because of the proximity of the stricture to the carina and the potential for restenosis. The shortness of the stenosed segment, the good condition of the lung distal to the stenosis, and the accessibility of the obstructed bronchus through the pennanent tracheostomy prompted the use of mechanical bronchial dilation once the inflammation was controlled. Persistent endobronchial obstruction may become more common in patients with Wegener's granulomatosis who now have an increase in their life expectancy. We suggest the following: (1) that radiation therapy can be beneficial when bronchial obstruction secondary to inHamed tissue persists in the absence of infection and despite treabnent with steroids and cyclophosphamide; and, (2) that repeated mechanical bronchial dilation can relieve bronchial obstruction secondary to residual scar tissue in patients with Wegener's granulomatosis. ACKNOWLEDGMENTS : We would lfke to express our appreciation to L. A. Von Behren, M.D., and Jacobo Wortsman, M.D.... F.A.C.P., for reviewing this paper and Ms. Helen Shelaon for assistance in the preparation of this manuscript. REFERENCES
DISCUSSION
The clinical manifestations, the biopsy Bndings, and the positive response to treatment with steroids and cyclophosphamide of our patient were the expected findings for Wegener's granulomatosis.3 ' 5 However, the persistence of a local inflammation followed by scarring which was sufficient to obstruct a major airway was an unexpected complication. Radiation therapy has been reported as effective treabnent for nasal Wegener's granulomatosts.s-" The resolution of the signs of inflammation in the bronchus and of the atelectasis which had persisted despite prolonged treatment with prednisone and cyclophosphamide suggests that the radiation therapy was effective in our patient. Spontaneous resolution of disease or response to other treabnents seemed less likely causes for the improvement. Resection has been employed to manage pulmonary consolidation in patients with Wegener's granulomatosis." However, a pneumonectomy seemed unwarranted in the absence of active disease. Resection of the stenosed bronchial segment was not attempted
610 EAGLETON ET AL
1 Maguire R, Fauci AS, Doppman JL, et al: Unusual radiographic features of Wegener's granulomatosis. Am J RoentgenoI130 :233-238,1978 2 Landman S, Burgener F : Pulmonary manifestations in Wegener's granulomatosis. Am J Roentgenol 122:750-757, 1974 3 Pritchard MH, Gow PG: Wegener's granulomatosis presenting as rheumatoid arthritis (two cases) . Proc Roy Soc Med 69:501-504, 1976 4 DeRemee RA, McDonald TJ, Doles DT : Wegener's granulomatosis-anatomic correlates, a proposed classification. Mayo Clin Proc 51:777-781,1976 5 Fauci AS, Haynes BF, Katz P: The spectrum of vasculitis, clinical, pathologic, immunologic, and therapeutic considerations. Ann Intern Med 89:660-676, 1978 6 Walton EW: Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Br Med J 266:265-270,1968 7 Singh MM, Stokes JF , Drury RAB, et al: The natural history of malignant granuloma of the nose. Lancet 1:401403, 1968 8 Landman S, Burgener F : Pulmonary manifestations in Wegener's granulomatosis. Am J Roentgenol Rad Ther Nucl Med 122:750-757, 1974
CHEST, 76: 5, NOVEMBER, 1979
COMMENTS
Radiation Therapy and Mechanical Dilation of Endobronchial Obstruction Secondary to Wegener's Granulomatosis* Lanie E. Eagleton, M.D., F.C.C.P.;··
This case illustrates a very unusual pulmonary manifestation of sarcoidosis, with bilateral pleuritic pain as the initial symptom and the presence of a bloody pleural effusion. Although we have not obtained histological demonstration of the pleural involvement by the granulomatous process, several lines of evidence support the diagnosis of pleural sarcoidosis. First, we found no other cause of bloody pleural effusion, such as tuberculosis, viral infection, pulmonary embolism, or neoplasm, and biopsy of mediastinal lymph nodes showed lesions typically seen in sarcoidosis. Second, the pleural effusion, as seen by repeated chest roentgenograms, remained stable over a period of four months, whereas it disappeared within a few days under prednisone therapy and did not recur in the ensuing three months. Finally, the treatment resulted in marked improvement of parenchymal infiltrates and of pulmonary function tests; the evolution of the lung mechanical properties under steroid therapy was identical to the pattern observed in patients with well-established sarcoidosis." In conclusion, sarcoidosis should be included in the differential diagnosis of bloody pleural effusion.
Richa,d B. Boshe« MD.;t
Anthony Hawe, M.B., Ch.B., F.R.C.S.;t and Richa,d T. BiUnsky, M.D.t
A patient with Wegener's granulomatosis, which was otherwise weD controDed with steroids an' cyelophosphamide, bad persistent total obstruction of an in8amed left maiDstem bronchus. The inflammation resolved, and file hmg expanded following radiation therapy• However, Intermittent atelectasis and pneumonia occurred distal to a residual short stricture. Repeated dilation endoscopicaDy with Plummer boogies bas proven effective In maintaining good ventilation and preventiDI recurrence of the atelectasis and pneumonia in the left lung for 18 months. secondary to endobronchial obA telectasis However, struction in Wegener's can occur
granulomatosis.t-s
there is little precedent in the literature to aid in the management of this complication. We report the successful management of recurrent bronchial obstroction with the combination of cyclophosphamide, prednisone and local radiation to control inflammation and repeated mechanical bronchial dilation to maintain patency of the airway. CASE REPORT
1 Case Records of the Massachusetts General Hospital (Case 1-1979). N Engl J Med 300:28-37, 1979 2 Mayock RL, Bertrand P, Morrison CE: Manifestations of sarcoidosis: Analysis of 145 patients, with a review of nine series selected hom the literature. Am J Med 35:67-89, 1963 3 Beekman ]F, Zimmet SM, Chun BK, et a1: Spectrum of pleural involvement of sarcoidosis. Arch Intern Med 136: 323-330, 1976 4 Wilen SB, Rabinowitz IG, Ulreich S, et al: Pleural Involvement in sarcoidosis. Am J Moo57:200-209, 1974 5 Sharma PO: Sarcoidosis: Unusual pulmonary manifestations. Postgrad Moo: 61:67-73, 1977 6 Chusid EL, Siltzbach LE: Sarcoidosis of the pleura. Ann Intern Med 81:190-194, 1974 7 Berte SI, pfotenhauer MA: Massive pleural effusion in sarcoidosis. Am Rev R~ir Dis 80:251-254, 1962 8 Littner MR, Schachter EN, Putman CE, et al: The clinical assessment of roentgenographically atypical pulmonary sarcoidosis. Am J Med 62:361-367, 1977 9 De Troyer A, Yernault JC, Diercla P, et al: Lung and airway mechanics in early pulmonary sarcoidosis. Bull Europ Physiopathol Respir 14:299-310, 1978
CHEST, 76: 5, NOVEMBER, 1979
This 35-year-old woman had persistent rhinitis, sinusitis, otitis, and arthritis which was treated with gold and steroids until 1969 when she developed glomerulonephritis. Wegener's granulomatosis was diagnosed based upon several biopsies showing the following: "vasculitis with fibrinoid necrosis in skin and muscle; acute and chronic inflammation with multinucleated giant cells in nasal mucosa; and proliferative glomerulonephritis and vasculitis in the kidney. The creatinine clearance level improved from 50 to 110 L/24 hours following an increase in the steroid dose. In 1971, she had granulomatous tracheitis causing subglottic stenosis. This was repaired surgically in 1975. She had fleeting pulmonary nodules at that time. In May 1976, she had acute dyspnea and severe chest pain concomitant with atelectasis of the left lung. A tracheostomy, currently maintained with a Kistner plastic tracheostomy tube, was made to facilitate bronchoscopy. The bronchial mucosa was erythematous, edematous, and very friable. The left main bronchus was occluded with granulation tissue; the right main bronchus was 3 mm in diameter. She was treated with cyclophosphamide and prednisone. The lung re-ex·From the Departments of Medicine and Surgery, Southern Dlinois University School of Medicine, Springfield, IL. ••Assistant Professor.
tlnstructor.
tClinical Associate Professor. Reprint reqfJe8ta: Dr. Eagleton, SIU School of Medicine, PO BO% 3926, Springfield, IUinoiB 62708
WEGENER'S GRANULOMATOSIS •
FIGURE 1. (A) (left) Carina in January 1977. The right mainstem bronchus is approximately 6 rom in diameter. Mucus is plugging "pit-like" orifice of the left mainstem bronchus on the left. ( B) (center) Left mainstem bronchus after suctioning free mucus. Mucosa is normal and orifice is approximately 1 mm in diameter. (C) (right) Carina following mechanical dilation in March 1977. Orifice of left mainstem bronchus is now approximately 3 rom in diameter.
panded three weeks later, but collapsed again in August 1976 when the left main bronchus again occluded with inflamed tissue. Because of persistent atelectasis one month later, radiation (2,000 rads) was administered to the trachea and bronchi. Two weeks after completion of this treatment, the lung re-expanded. An additional 2,000 rads were given. In January 1977, there were brief recurrences of atelectasis secondary to mucous obstruction. The mucosa of the trachea and bronchi appeared normal. However, the left mainstem bronchus had a l-mm orifice which was obstructed with mucus (Fig 1) . As demonstrated by tomography, the stenotic segment of bronchus had a length of only 1 em. Poor ventilation and chest pain persisted. In March, the left lung was consolidated as a result of a pneumococcal pneumonia. One week later, dilation of the left mainstem bronchus was begun with Plummer esophageal bougies. During the ensuing two years, this mechanical dilation has been repeated °ten times at intervals of one to six months. There has been no recurrence of atelectasis or pneumonia in the left lung. Treatment with cyclophosphamide has been continued. Currently her creatinine clearance level and forced vital capacity (FVC) are within normal limits. The FEVt/FVC ratio is .62. She is a physically active housekeeper and mother.
because of the proximity of the stricture to the carina and the potential for restenosis. The shortness of the stenosed segment, the good condition of the lung distal to the stenosis, and the accessibility of the obstructed bronchus through the pennanent tracheostomy prompted the use of mechanical bronchial dilation once the inflammation was controlled. Persistent endobronchial obstruction may become more common in patients with Wegener's granulomatosis who now have an increase in their life expectancy. We suggest the following: (1) that radiation therapy can be beneficial when bronchial obstruction secondary to inHamed tissue persists in the absence of infection and despite treabnent with steroids and cyclophosphamide; and, (2) that repeated mechanical bronchial dilation can relieve bronchial obstruction secondary to residual scar tissue in patients with Wegener's granulomatosis. ACKNOWLEDGMENTS : We would lfke to express our appreciation to L. A. Von Behren, M.D., and Jacobo Wortsman, M.D.... F.A.C.P., for reviewing this paper and Ms. Helen Shelaon for assistance in the preparation of this manuscript. REFERENCES
DISCUSSION
The clinical manifestations, the biopsy Bndings, and the positive response to treatment with steroids and cyclophosphamide of our patient were the expected findings for Wegener's granulomatosis.3 ' 5 However, the persistence of a local inflammation followed by scarring which was sufficient to obstruct a major airway was an unexpected complication. Radiation therapy has been reported as effective treabnent for nasal Wegener's granulomatosts.s-" The resolution of the signs of inflammation in the bronchus and of the atelectasis which had persisted despite prolonged treatment with prednisone and cyclophosphamide suggests that the radiation therapy was effective in our patient. Spontaneous resolution of disease or response to other treabnents seemed less likely causes for the improvement. Resection has been employed to manage pulmonary consolidation in patients with Wegener's granulomatosis." However, a pneumonectomy seemed unwarranted in the absence of active disease. Resection of the stenosed bronchial segment was not attempted
610 EAGLETON ET AL
1 Maguire R, Fauci AS, Doppman JL, et al: Unusual radiographic features of Wegener's granulomatosis. Am J RoentgenoI130 :233-238,1978 2 Landman S, Burgener F : Pulmonary manifestations in Wegener's granulomatosis. Am J Roentgenol 122:750-757, 1974 3 Pritchard MH, Gow PG: Wegener's granulomatosis presenting as rheumatoid arthritis (two cases) . Proc Roy Soc Med 69:501-504, 1976 4 DeRemee RA, McDonald TJ, Doles DT : Wegener's granulomatosis-anatomic correlates, a proposed classification. Mayo Clin Proc 51:777-781,1976 5 Fauci AS, Haynes BF, Katz P: The spectrum of vasculitis, clinical, pathologic, immunologic, and therapeutic considerations. Ann Intern Med 89:660-676, 1978 6 Walton EW: Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Br Med J 266:265-270,1968 7 Singh MM, Stokes JF , Drury RAB, et al: The natural history of malignant granuloma of the nose. Lancet 1:401403, 1968 8 Landman S, Burgener F : Pulmonary manifestations in Wegener's granulomatosis. Am J Roentgenol Rad Ther Nucl Med 122:750-757, 1974
CHEST, 76: 5, NOVEMBER, 1979