The Many Faces of Sarcoidosis Luther B. Adair, MD, James E. Smith, MD, and Harry C. Press, Jr., MD Boston, Massachusetts and Washington, D.C.
This is a radiographic exhibit demonstrating some of the many ways that pulmonary sarcoidosis can present. In addition to the classical stages (I-IV), sarcoidosis can sometimes present atypically. Some of these forms of sarcoid disease have been included in this exhibit. Sarcoidosis, also known as Boeck's sarcoid and Besnier-Boeck-Schaumann's disease is of unknown etiology. It is characterized pathologically by non-caseating granulomas whose presence in many organs, including the lung, liver, spleen, lymph node, skin, and bone create a clinical and roentgenographic complex usually indicative of the disease. Sarcoidosis has a world-wide distribution. However, most large series have been reported from Scandinavia, England, and the United States. It appears to be more prevalent in rural areas, a distribution that has been particularly noted in the southeastern
Exhibited at the annual convention of the National Medical Association, Nashville, Tenn, Aug, 1976. From Massachusetts General Hospital and Howard University Hospital. Requests for reprints should be addressed to Dr. Harry C. Press, Jr., Department of Radiology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, D.C. 20060.
United States and in Switzerland. Epidemiologic studies on military personnel suggest that the greatest concentration of sarcoidosis in the United States occurs in the eastern - particularly the southeastern - portion of the country, with an incidence in blacks 10 to 17 times greater than in American whites. The lung and hilar lymph nodes are most commonly involved, ie, in 80 to 90 percent of the cases. This strongly suggests that the etiologic agent(s) enter the body via the lung, probably by inhalation, and dissemination to other organs and tissues follows.
Stage 1. Lymph Node Enlargement without Pulmonary Abnormality Bilateral hilar lymph-node enlargement occurs in 75-90 percent of patients with sarcoidosis, approximately
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 10, 1977
equal numbers of whom have or do not have diffuse parenchymal disease. Node enlargement usually is localized to the paratracheal, tracheobronchial, and bronchopulmonary groups and is almost always symmetrical bilaterally (Figure 1). The contours of the outer borders of enlarged hila usually are lobulated, particularly on the right side. Lymph node calcification is unusual. Paratracheal node enlargement is more evident on the right side than on the left and seldom if ever occurs without concomitant enlargement of hilar nodes. This bilaterally symmetrical hilar and paratracheal lymph-node enlargement contrasts sharply with the node enlargement of primary tuberculosis, which tends to be unilateral and less sharply demarcated. In lymphomas, enlargement tends to occur predominantly in the paratracheal group. When it involves the hilar nodes, the enlargement is predominantly unilateral and asymmetrical. Retrosternal node enlargement is also fairly common in lymphoma but rare in sarcoidosis. Characteristically, there is a relatively translucent space between the mass of nodes and the cardiovascular margin. This is more apparent on the right side, where the lung hilum normally is 715
4 Figure 1. PA chest film showing right paratracheal and bilateral hilar adenopathy.
better seen than on the left. In contrast, Hodgkin's disease, a frequent source of difficulty in differential diagnosis, is more likely to involve the more centrally situated nodes around the tracheal bifurcation and those in the hila. Often the mass of nodes tends to merge with the cardiovascular silhouette. Additionally, in sarcoidosis, the onset of diffuse lung disease is commonly associated with a dimunition in lymph node size or at least with cessation of their growth, a finding not observed in lymphoma. Seventy-five percent of sarcoid patients with hilar lymph node enlargement show complete resolution of the enlarged nodes.
Stage I1. Combined Diffuse Pulmonary Disease and Lymph Node Enlargement When the two coexist, diffuse pulmonary disease and lymphnode en716
Figure 2. PA chest film showing paratracheal and hilar adenopathy with diffuse reticulonodular densities in both lung fields.
largement differ greatly in their temporal relationship in various patients. Diffuse pulmonary disease usually appears when hilar node enlargement is present, although that condition may be regressing (Figure 2). Node enlargement may disappear and be replaced by diffuse pulmonary involvement, either concurrently or several years later. It may remain and diffuse pulmonary involvement may be superimposed.1
Stage 111. Diffuse Pulmonary Disease without Lymph Node Enlargement Approximately 25 percent of all sarcoid patients present this way. The pulmonary abnormality is diffuse and is most often evenly distributed throughout the lung (Figure 3). The roentgenographic pattern usually is "reticulonodular" but may vary from purely reticular to purely nodular. The
Figure 3. PA chest film showing multiple nodular densities throughout both lung fields.
nodules are usually discrete and small (1 to 3 mm).2 Rarely, as in Figure 3, patients may present with larger nodules. Metastatic disease, as well as others, must be excluded in these unusual cases.
Stage IV. Pulmonary Fibrosis or Honeycomb Lung When pulmonary changes have been present for more than two years, resolution is the exception rather than the rule. Scarring is rather coarse, in the form of irregular linear strands extending outward from the hila toward the periphery (Figure 4), and is commonly more uneven in distribution than in the recticulonodular pattem which is characteristic of the active state. It is associated with welldefined structural changes in the lung, including bleb or bulla formation, bronchiectasis, and general emphy-
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 10, 1977
Figure 4. PA chest film showing fibrosis and "honeycombing."
sema.3 When fibrosis and emphysema are severe, changes in the heart and pulmonary vasculature are typical of those due to pulmonary hypertension and cor pulmonale. The honeycomb lung is actually an end-stage scarring of the lung parenchyma. Many diseases may produce this end-stage process. Some of the more common causes of the honeycomb lung include cystic bronchi-
ectasis, histiocytosis (especially eosinophilic granuloma), idiopathic interstitial fibrosis (Hamman-Rich), pneumoconiosis, and sarcoidosis. Other somewhat uncommon causes are amyloidosis, chemical inhalation (late stage), desquamative interstitial pneumonitis (DIP), chronic or recurrent drug sensitivity (as to nitrofurantion hexamethonium or busulfan), Gaucher's disease, lipoid pneumonia (mineral and aspiration), lymphangiomyomatosis, 02 toxcity, mucoviscidosis, neurofibromatosis, rheumatoid lung, sclerod e rma, dermatomyositis, recurrent sensitivity pneumonitis (farmer's lung, bagassosis), and tuberous sclerosis.
igure 5. PA chest film showing right hilar adenopathy.
Unilateral Hilar Adenopathy Unilateral node enlargement occurs in one to three percent of reported cases (Figure 5). Our series at Howard University Hospital indicates that it may be higher (up to 15 percent). Unilateral hilar adenopathy is a rare finding in sarcoidosis.1 ,4-6 Tuberculosis, carcinoma, and lymphoma should be considered in the differential diagnosis.
Figure 6. PA chest film showing patchy alveolar infiltrates in both lung fields.
Summary These are some of the many ways that pulmonary sarcoid disease can manifest itself radiographically. In addition to the classical mode of presentation, sarcoid may present in other ways, and, if clinically suspected, this diagnosis should be entertained in the presence of unilateral hilar adenopathy, pulmonary nodules, or alveolar disease. References
Alveolar Sarcoidosis Sarcoidosis is a disease of the pulmonary interstitium and the term alveolar sarcoidosis (Figure 6) is a roentgenographic diagnosis based on certain proposed criteria. It appears that the alveolar radiographic pattern may represent a secondary nonspecific response of the lung to the primary interstitial injury and not intra-alveolar granulomas.7
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 10, 1977
1. Fraser RG, Pare JAP (eds): Diagnosis of Disease of the Chest. Philadelphia, WB Saunders, 1970, p 552 2. Felson B: Fundamentals of Chest Roentgenology. Philadelphia, WB Saunders, 1970, p 127 3. Reed JC, Reeder MM: Honeycomb lung (interstitial fibrosis). JAMA 231:646-647, 1975 4. Brill PW, Bloch C, Peck HM: Pediatric radiologic notes. Mt Sinai J Med NY 4(3)488-490, 1974 5. Sitzbach LE, Greenberg GM: Childhood sarcoidosis - a study of 18 patients. N Engl J Med 279:1239-1245, 1968 6. Shmitt E, Appleman A, Threatt B: Sarcoidosis in children. Radiology 106:621-625, 1973 7. Sahn SA, Schwartz MlI, Lakshminarayan 5: Sarcoidosis: The significance of an acinar pattern on chest roentgenogram. Chest 65(6):684-687, 1974
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This is a radiographic exhibit demonstrating some of the many ways that pulmonary sarcoidosis can present. In addition to the classical stages (I-IV), sarcoidosis can sometimes present atypically. Some of these forms of sarcoid disease have been included in this exhibit. Sarcoidosis, also known as Boeck's sarcoid and Besnier-Boeck-Schaumann's disease is of unknown etiology. It is characterized pathologically by non-caseating granulomas whose presence in many organs, including the lung, liver, spleen, lymph node, skin, and bone create a clinical and roentgenographic complex usually indicative of the disease. Sarcoidosis has a world-wide distribution. However, most large series have been reported from Scandinavia, England, and the United States. It appears to be more prevalent in rural areas, a distribution that has been particularly noted in the southeastern
Exhibited at the annual convention of the National Medical Association, Nashville, Tenn, Aug, 1976. From Massachusetts General Hospital and Howard University Hospital. Requests for reprints should be addressed to Dr. Harry C. Press, Jr., Department of Radiology, Howard University Hospital, 2041 Georgia Avenue NW, Washington, D.C. 20060.
United States and in Switzerland. Epidemiologic studies on military personnel suggest that the greatest concentration of sarcoidosis in the United States occurs in the eastern - particularly the southeastern - portion of the country, with an incidence in blacks 10 to 17 times greater than in American whites. The lung and hilar lymph nodes are most commonly involved, ie, in 80 to 90 percent of the cases. This strongly suggests that the etiologic agent(s) enter the body via the lung, probably by inhalation, and dissemination to other organs and tissues follows.
Stage 1. Lymph Node Enlargement without Pulmonary Abnormality Bilateral hilar lymph-node enlargement occurs in 75-90 percent of patients with sarcoidosis, approximately
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 10, 1977
equal numbers of whom have or do not have diffuse parenchymal disease. Node enlargement usually is localized to the paratracheal, tracheobronchial, and bronchopulmonary groups and is almost always symmetrical bilaterally (Figure 1). The contours of the outer borders of enlarged hila usually are lobulated, particularly on the right side. Lymph node calcification is unusual. Paratracheal node enlargement is more evident on the right side than on the left and seldom if ever occurs without concomitant enlargement of hilar nodes. This bilaterally symmetrical hilar and paratracheal lymph-node enlargement contrasts sharply with the node enlargement of primary tuberculosis, which tends to be unilateral and less sharply demarcated. In lymphomas, enlargement tends to occur predominantly in the paratracheal group. When it involves the hilar nodes, the enlargement is predominantly unilateral and asymmetrical. Retrosternal node enlargement is also fairly common in lymphoma but rare in sarcoidosis. Characteristically, there is a relatively translucent space between the mass of nodes and the cardiovascular margin. This is more apparent on the right side, where the lung hilum normally is 715
4 Figure 1. PA chest film showing right paratracheal and bilateral hilar adenopathy.
better seen than on the left. In contrast, Hodgkin's disease, a frequent source of difficulty in differential diagnosis, is more likely to involve the more centrally situated nodes around the tracheal bifurcation and those in the hila. Often the mass of nodes tends to merge with the cardiovascular silhouette. Additionally, in sarcoidosis, the onset of diffuse lung disease is commonly associated with a dimunition in lymph node size or at least with cessation of their growth, a finding not observed in lymphoma. Seventy-five percent of sarcoid patients with hilar lymph node enlargement show complete resolution of the enlarged nodes.
Stage I1. Combined Diffuse Pulmonary Disease and Lymph Node Enlargement When the two coexist, diffuse pulmonary disease and lymphnode en716
Figure 2. PA chest film showing paratracheal and hilar adenopathy with diffuse reticulonodular densities in both lung fields.
largement differ greatly in their temporal relationship in various patients. Diffuse pulmonary disease usually appears when hilar node enlargement is present, although that condition may be regressing (Figure 2). Node enlargement may disappear and be replaced by diffuse pulmonary involvement, either concurrently or several years later. It may remain and diffuse pulmonary involvement may be superimposed.1
Stage 111. Diffuse Pulmonary Disease without Lymph Node Enlargement Approximately 25 percent of all sarcoid patients present this way. The pulmonary abnormality is diffuse and is most often evenly distributed throughout the lung (Figure 3). The roentgenographic pattern usually is "reticulonodular" but may vary from purely reticular to purely nodular. The
Figure 3. PA chest film showing multiple nodular densities throughout both lung fields.
nodules are usually discrete and small (1 to 3 mm).2 Rarely, as in Figure 3, patients may present with larger nodules. Metastatic disease, as well as others, must be excluded in these unusual cases.
Stage IV. Pulmonary Fibrosis or Honeycomb Lung When pulmonary changes have been present for more than two years, resolution is the exception rather than the rule. Scarring is rather coarse, in the form of irregular linear strands extending outward from the hila toward the periphery (Figure 4), and is commonly more uneven in distribution than in the recticulonodular pattem which is characteristic of the active state. It is associated with welldefined structural changes in the lung, including bleb or bulla formation, bronchiectasis, and general emphy-
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 10, 1977
Figure 4. PA chest film showing fibrosis and "honeycombing."
sema.3 When fibrosis and emphysema are severe, changes in the heart and pulmonary vasculature are typical of those due to pulmonary hypertension and cor pulmonale. The honeycomb lung is actually an end-stage scarring of the lung parenchyma. Many diseases may produce this end-stage process. Some of the more common causes of the honeycomb lung include cystic bronchi-
ectasis, histiocytosis (especially eosinophilic granuloma), idiopathic interstitial fibrosis (Hamman-Rich), pneumoconiosis, and sarcoidosis. Other somewhat uncommon causes are amyloidosis, chemical inhalation (late stage), desquamative interstitial pneumonitis (DIP), chronic or recurrent drug sensitivity (as to nitrofurantion hexamethonium or busulfan), Gaucher's disease, lipoid pneumonia (mineral and aspiration), lymphangiomyomatosis, 02 toxcity, mucoviscidosis, neurofibromatosis, rheumatoid lung, sclerod e rma, dermatomyositis, recurrent sensitivity pneumonitis (farmer's lung, bagassosis), and tuberous sclerosis.
igure 5. PA chest film showing right hilar adenopathy.
Unilateral Hilar Adenopathy Unilateral node enlargement occurs in one to three percent of reported cases (Figure 5). Our series at Howard University Hospital indicates that it may be higher (up to 15 percent). Unilateral hilar adenopathy is a rare finding in sarcoidosis.1 ,4-6 Tuberculosis, carcinoma, and lymphoma should be considered in the differential diagnosis.
Figure 6. PA chest film showing patchy alveolar infiltrates in both lung fields.
Summary These are some of the many ways that pulmonary sarcoid disease can manifest itself radiographically. In addition to the classical mode of presentation, sarcoid may present in other ways, and, if clinically suspected, this diagnosis should be entertained in the presence of unilateral hilar adenopathy, pulmonary nodules, or alveolar disease. References
Alveolar Sarcoidosis Sarcoidosis is a disease of the pulmonary interstitium and the term alveolar sarcoidosis (Figure 6) is a roentgenographic diagnosis based on certain proposed criteria. It appears that the alveolar radiographic pattern may represent a secondary nonspecific response of the lung to the primary interstitial injury and not intra-alveolar granulomas.7
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 10, 1977
1. Fraser RG, Pare JAP (eds): Diagnosis of Disease of the Chest. Philadelphia, WB Saunders, 1970, p 552 2. Felson B: Fundamentals of Chest Roentgenology. Philadelphia, WB Saunders, 1970, p 127 3. Reed JC, Reeder MM: Honeycomb lung (interstitial fibrosis). JAMA 231:646-647, 1975 4. Brill PW, Bloch C, Peck HM: Pediatric radiologic notes. Mt Sinai J Med NY 4(3)488-490, 1974 5. Sitzbach LE, Greenberg GM: Childhood sarcoidosis - a study of 18 patients. N Engl J Med 279:1239-1245, 1968 6. Shmitt E, Appleman A, Threatt B: Sarcoidosis in children. Radiology 106:621-625, 1973 7. Sahn SA, Schwartz MlI, Lakshminarayan 5: Sarcoidosis: The significance of an acinar pattern on chest roentgenogram. Chest 65(6):684-687, 1974
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