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Right Apical Opacification on a Chest Radiograph and Chest Pain
roentgenogram of the month Right Apical Opacification on a Chest Radiograph and Chest Pain* Jonathan Spratt; Sylvia Worthy, MB; Andrew Grainger; and Stephen Bourke, MD
(CHEST 2003; 124:1143–1144)
55-year-old man presented to the hospital on A several occasions with “atypical” chest pain some-
times consistent with angina, with normal ECG findings and cardiac enzyme levels. The only risk factor for ischemic heart disease was smoking 10 to 20 cigarettes a day. His medical history included hematuria, with normal IV urogram and cystoscopic findings. He was unable to perform an exercise test due to severe diffuse “arthritis” (previous radiographic confirmation of lumbar and cervical osteoarthritis), and results of a subsequent dipyridamole myocardial perfusion scan suggested mild ischemic heart disease. He was started on treatment with aspirin, atenolol, and glyceryl trinitrate spray. A chest radiograph (Fig 1) was performed that revealed a new hazy opacity in the right upper zone, and referral to a chest physician was made. Subsequent bronchoscopy did not show any endobronchial lesion, and test findings of BAL fluid for cytology, bacteria, mycobacteria, fungi, viruses, and Pneumocystis carinii were negative. CT of the thorax showed no evidence of a lung mass, and he was assumed to have had a transient area of infective consolidation. However, another chest radiograph revealed persistent unchanged right-upper-zone opacification. Reassessment of the patient revealed that his chest pain was, at least sometimes, related to tenderness over the right upper anterior chest wall. A full blood count, levels of calcium phosphate, alkaline phosphatase, and rheumatoid factor, and an autoantibody screen were performed and found to be normal. The erythrocyte *From the Departments of Respiratory Medicine (Dr. Bourke) and Radiology (Drs. Spratt and Worthy), Royal Victoria Infirmary, Newcastle-on-Tyne; the Department of Radiology (Dr. Grainger), Freeman Hospital, Newcastle-on-Tyne, UK. Manuscript received January 29, 2002; revision accepted March 18, 2002. Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail: [email protected]). Correspondence to: Jonathan Spratt, FRCS, FRCR, Department of Radiology, University Hospital of North Durham, North Rd, Durham DH1 5TW, UK; e-mail: [email protected] www.chestjournal.org
Figure 1. Posteroanterior chest radiograph demonstrating increased opacification in the right upper zone.
sedimentation rate was mildly elevated at 14 mm in the first hour. A bone scan revealed raised uptake in the right sternoclavicular joint, the left second rib, and the body and manubrium of the sternum (Fig 2); the remainder of the skeleton was normal. What is the diagnosis?
Figure 2. Isotope bone scan demonstrating raised uptake in the right sternoclavicular joint, the left second rib, and the sternum. CHEST / 124 / 3 / SEPTEMBER, 2003
1143
Diagnosis: Sternocostoclavicular hyperostosis The chest CT study was reviewed, and bone overgrowth and adjacent soft-tissue ossification of the right clavicle, anterior portion of the first rib, and sternum typical of sternocostoclavicular hyperostosis were identified (Fig 3). The increased opacity shown on the chest radiograph raised the suspicion of right apical lung cancer. The CT differential diagnosis includes Paget disease (soft-tissue ossification and normal alkaline phosphatase level unlikely), chronic osteomyelitis, and osteitis condensans. Sternocostoclavicular hyperostosis affects men more frequently than women, especially Japanese, in the fourth to sixth decades of life. The etiology is unknown. Clinical findings include pain, swelling, tenderness, and local heat in the anterior upper chest, bilateral changes predominating. Erythrocyte sedimentation rate, ␣2 globulin, and C-reactive protein levels may be elevated. Leukocytosis is typically absent, and serologic testing for rheumatoid factor and human leukocyte antigen B27 gives normal results. Osseous overgrowth may lead to occlusion of the subclavian veins with considerable edema1 or brachial plexopathy. Ossification of a varying degree involves the costoclavicular ligament, inferior margin of the clavicle, and superior margin of the first rib. Hyperostosis of the sternum, clavicle, and upper ribs and sometimes around the manubriosternal junction is evident.2– 4 Additional changes may occur in the vertebral column, which include syndesmophytes, para-articular osseous bridging, and erosions that resemble ankylosing spondylitis including the sacroiliac joints, diffuse idiopathic skeletal hyperostosis, or psoriatic
spondylopathy. “Ivory vertebrae” resembling metastases may also be seen. Swelling, tenderness, and pain on movement are documented in extra-axial locations, but radiographic findings are usually only of soft-tissue swelling, without erosive change. Periosteal proliferation and sclerosis have been identified in long tubular bones.5 Sternocostoclavicular hyperostosis is one of a group of conditions that feature hyperostosis, often associated with cutaneous abnormalities within the SAPHO syndromes (synovitis, acne, pustulosis, hyperostosis, osteitis). These syndromes include chronic recurrent multifocal osteomyelitis that is seen in the pediatric population and may show similar changes of hyperostosis. Cutaneous changes may be seen in sternocostoclavicular hyperostosis in the form of palmoplantar pustulosis, which is present in 30 to 50% of patients, typically within 2 years before or after but more often simultaneously with the skeletal manifestations.6 To further confuse potential etiologies, identical lesions are present with chronic recurrent multifocal osteomyelitis, another condition that causes clavicular hyperostosis.7 Histologic examination from bone biopsies of ossified thoracic masses resemble Paget disease with dense fibrosis and bone formation, granulation tissue, and round cell infiltrates.8 The majority of cultures are sterile. Sternocostoclavicular hyperostosis has a protracted course with periods of exacerbation and remission with slow progression of the musculoskeletal changes. A favorable clinical response to antibiotics and prostaglandin inhibitors has been observed. The chest radiograph appearances may cause confusion! References
Figure 3. Thoracic CT demonstrating bone overgrowth of the sternum and soft-tissue ossification of the sternoclavicular joints bilaterally. 1144
1 van Holsbeeck M, Martel W, Dequeker J, et al. Soft tissue involvement, mediastinal pseudotumour and venous thrombosis in pustulotic arthro-osteitis. Skeletal Radiol 1989; 18:1– 8 2 Resnick D. Sternocostoclavicular hyperostosis. AJR Am J Roentgenol 1980; 135:1278 –1280 3 Resnick D, Vint V, Poteshman NL. Sternocostoclavicular hyperostosis: a report of three new cases. J Bone Joint Surg Am 1981; 63:1329 –1332 4 Sartoris DJ, Schreimann JS, Kerr R, et al. Sternocostoclavicular hyperostosis: a review and report of 11 cases. Radiology 1986; 158:125–128 5 Patterson AC, Bentley-Corbett K. Pustulotic arthro-osteitis. J Rheumatol 1985; 12:611⫺614 6 Sonozaki H, Kawashima M, Hongo O, et al. Incidence of arthro-osteitis in patients with pustulosis palmaris et plantaris. Ann Rheum Dis 1981; 40:554 –557 7 Laxer RM, Shore AD, Manson D, et al. Chronic recurrent multifocal osteomyelitis and psoriasis: a report of a new association and review of related disorders. Semin Arthritis Rheum 1988; 17:260 –270 8 Lagier R, Arroyo J, Fallet GH. Sternocostoclavicular hyperostosis: radiological and pathological study of a specimen with ununited clavicular fracture. Pathol Res Pract 1986; 181: 596 – 603 Roentgenogram of the month
(CHEST 2003; 124:1143–1144)
55-year-old man presented to the hospital on A several occasions with “atypical” chest pain some-
times consistent with angina, with normal ECG findings and cardiac enzyme levels. The only risk factor for ischemic heart disease was smoking 10 to 20 cigarettes a day. His medical history included hematuria, with normal IV urogram and cystoscopic findings. He was unable to perform an exercise test due to severe diffuse “arthritis” (previous radiographic confirmation of lumbar and cervical osteoarthritis), and results of a subsequent dipyridamole myocardial perfusion scan suggested mild ischemic heart disease. He was started on treatment with aspirin, atenolol, and glyceryl trinitrate spray. A chest radiograph (Fig 1) was performed that revealed a new hazy opacity in the right upper zone, and referral to a chest physician was made. Subsequent bronchoscopy did not show any endobronchial lesion, and test findings of BAL fluid for cytology, bacteria, mycobacteria, fungi, viruses, and Pneumocystis carinii were negative. CT of the thorax showed no evidence of a lung mass, and he was assumed to have had a transient area of infective consolidation. However, another chest radiograph revealed persistent unchanged right-upper-zone opacification. Reassessment of the patient revealed that his chest pain was, at least sometimes, related to tenderness over the right upper anterior chest wall. A full blood count, levels of calcium phosphate, alkaline phosphatase, and rheumatoid factor, and an autoantibody screen were performed and found to be normal. The erythrocyte *From the Departments of Respiratory Medicine (Dr. Bourke) and Radiology (Drs. Spratt and Worthy), Royal Victoria Infirmary, Newcastle-on-Tyne; the Department of Radiology (Dr. Grainger), Freeman Hospital, Newcastle-on-Tyne, UK. Manuscript received January 29, 2002; revision accepted March 18, 2002. Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail: [email protected]). Correspondence to: Jonathan Spratt, FRCS, FRCR, Department of Radiology, University Hospital of North Durham, North Rd, Durham DH1 5TW, UK; e-mail: [email protected] www.chestjournal.org
Figure 1. Posteroanterior chest radiograph demonstrating increased opacification in the right upper zone.
sedimentation rate was mildly elevated at 14 mm in the first hour. A bone scan revealed raised uptake in the right sternoclavicular joint, the left second rib, and the body and manubrium of the sternum (Fig 2); the remainder of the skeleton was normal. What is the diagnosis?
Figure 2. Isotope bone scan demonstrating raised uptake in the right sternoclavicular joint, the left second rib, and the sternum. CHEST / 124 / 3 / SEPTEMBER, 2003
1143
Diagnosis: Sternocostoclavicular hyperostosis The chest CT study was reviewed, and bone overgrowth and adjacent soft-tissue ossification of the right clavicle, anterior portion of the first rib, and sternum typical of sternocostoclavicular hyperostosis were identified (Fig 3). The increased opacity shown on the chest radiograph raised the suspicion of right apical lung cancer. The CT differential diagnosis includes Paget disease (soft-tissue ossification and normal alkaline phosphatase level unlikely), chronic osteomyelitis, and osteitis condensans. Sternocostoclavicular hyperostosis affects men more frequently than women, especially Japanese, in the fourth to sixth decades of life. The etiology is unknown. Clinical findings include pain, swelling, tenderness, and local heat in the anterior upper chest, bilateral changes predominating. Erythrocyte sedimentation rate, ␣2 globulin, and C-reactive protein levels may be elevated. Leukocytosis is typically absent, and serologic testing for rheumatoid factor and human leukocyte antigen B27 gives normal results. Osseous overgrowth may lead to occlusion of the subclavian veins with considerable edema1 or brachial plexopathy. Ossification of a varying degree involves the costoclavicular ligament, inferior margin of the clavicle, and superior margin of the first rib. Hyperostosis of the sternum, clavicle, and upper ribs and sometimes around the manubriosternal junction is evident.2– 4 Additional changes may occur in the vertebral column, which include syndesmophytes, para-articular osseous bridging, and erosions that resemble ankylosing spondylitis including the sacroiliac joints, diffuse idiopathic skeletal hyperostosis, or psoriatic
spondylopathy. “Ivory vertebrae” resembling metastases may also be seen. Swelling, tenderness, and pain on movement are documented in extra-axial locations, but radiographic findings are usually only of soft-tissue swelling, without erosive change. Periosteal proliferation and sclerosis have been identified in long tubular bones.5 Sternocostoclavicular hyperostosis is one of a group of conditions that feature hyperostosis, often associated with cutaneous abnormalities within the SAPHO syndromes (synovitis, acne, pustulosis, hyperostosis, osteitis). These syndromes include chronic recurrent multifocal osteomyelitis that is seen in the pediatric population and may show similar changes of hyperostosis. Cutaneous changes may be seen in sternocostoclavicular hyperostosis in the form of palmoplantar pustulosis, which is present in 30 to 50% of patients, typically within 2 years before or after but more often simultaneously with the skeletal manifestations.6 To further confuse potential etiologies, identical lesions are present with chronic recurrent multifocal osteomyelitis, another condition that causes clavicular hyperostosis.7 Histologic examination from bone biopsies of ossified thoracic masses resemble Paget disease with dense fibrosis and bone formation, granulation tissue, and round cell infiltrates.8 The majority of cultures are sterile. Sternocostoclavicular hyperostosis has a protracted course with periods of exacerbation and remission with slow progression of the musculoskeletal changes. A favorable clinical response to antibiotics and prostaglandin inhibitors has been observed. The chest radiograph appearances may cause confusion! References
Figure 3. Thoracic CT demonstrating bone overgrowth of the sternum and soft-tissue ossification of the sternoclavicular joints bilaterally. 1144
1 van Holsbeeck M, Martel W, Dequeker J, et al. Soft tissue involvement, mediastinal pseudotumour and venous thrombosis in pustulotic arthro-osteitis. Skeletal Radiol 1989; 18:1– 8 2 Resnick D. Sternocostoclavicular hyperostosis. AJR Am J Roentgenol 1980; 135:1278 –1280 3 Resnick D, Vint V, Poteshman NL. Sternocostoclavicular hyperostosis: a report of three new cases. J Bone Joint Surg Am 1981; 63:1329 –1332 4 Sartoris DJ, Schreimann JS, Kerr R, et al. Sternocostoclavicular hyperostosis: a review and report of 11 cases. Radiology 1986; 158:125–128 5 Patterson AC, Bentley-Corbett K. Pustulotic arthro-osteitis. J Rheumatol 1985; 12:611⫺614 6 Sonozaki H, Kawashima M, Hongo O, et al. Incidence of arthro-osteitis in patients with pustulosis palmaris et plantaris. Ann Rheum Dis 1981; 40:554 –557 7 Laxer RM, Shore AD, Manson D, et al. Chronic recurrent multifocal osteomyelitis and psoriasis: a report of a new association and review of related disorders. Semin Arthritis Rheum 1988; 17:260 –270 8 Lagier R, Arroyo J, Fallet GH. Sternocostoclavicular hyperostosis: radiological and pathological study of a specimen with ununited clavicular fracture. Pathol Res Pract 1986; 181: 596 – 603 Roentgenogram of the month