Pulmonary Manifestations Of Skeletal Disorders

Transcription

1 Pulmonary Manifestations Of Skeletal Disorders U. A. Saeed, MBBS FCPS, J. Nair, MBBS MD, R. Khosla, MD FRCR, K. Sayegh, MD FRCPC, J. Kosiuk, MD FRCPC, J. Taylor, MD FRCPC; Department of Radiology, McGill University Health Centre, Montreal, QC, CANADA. SPECIAL ACKNOWLEDGEMENT: Carl Frédéric Duchatellier, MD

2 I m coming t o get YOU!

3 DISCLOSURES NO RELEVANT DISCLOSURES

4 INTRODUCTION Lungs are involved in numerous skeletal disorders as: An isolated pulmonary manifestation An extra-osseous pulmonary feature of the skeletal disorder A complication of the underlying skeletal disease As a result of the treatment of the skeletal disorder

5 OBJECTIVE 1) Identification of the various pulmonary manifestations of skeletal disorders. 2) Correlation of clinical and characteristic pulmonary HRCT findings in various skeletal disorders to improve better understanding of these pathologies. 3) Discussion of the relevant differential diagnosis of each of these disorders.

6 SKELETAL DISORDERS UNDER DISCUSSION Rheumatoid Arthritis Scleroderma Hypertrophic Osteoarthropathy Osteopyte Induced Fibrosis Langerhans Cell Histiocytosis Erdheim- Chester Disease Systemic lupus erythematosis Ankylosing spondylitis

7 RHEUMATOID ARTHRITIS Following infections, pleuropulmonary involvement is the second most common cause of death in patients with Rheumatoid Arthritis, after infections. Significant prognostic value

8 RHEUMATOID ARTHRITIS PLEURA: - Effusion, Thickening, Empyema PARENCHYMA - ILD (commonly UIP) - Nodules - Caplan s Syndrome (RA+ Pulmonary nodules+ Coal Miners Pneumoconiosis) AIRWAY - Bronchiectasis, Cryptogenic oraganizing pneumonia, Follicular bronchiolitis

9 UIP type of lung fibrosis and pleural thickening in a patient with Rheumatoid Arthritis

10 SCLERODERMA Mediastinum -Dilated, patulous esophagus -Rare Esophageal cancer Parenchymal involvement - NSIP type of ILD -Pulmonary hypertension - Pnuemonitis aspiration infection

11 NSIP type of basal lung fibrosis and patulous esophagus in a patient of Scleroderma

12 HYPERTROPHIC OSTEOARTHROPATHY Periosteal reaction involving the diaphysis/ meta-diaphysis of long bones of distal extremities Clubbing - seen in patients with lung, liver and gastrointestinal disorders

13 HYPERTROPHIC OSTEOARTHROPATHY Bronchogenic Carcinoma Pulmonary Lymphoma Lung Abscess Bronchiectasis Pulmonary Metastases (especially osteosarcoma) Pleural Fibroma Mesothelioma

14 Right middle lobe mass. X-ray tibia and fibula shows diffuse, smooth periosteal reaction

15 OSTEOPHYTE INDUCED FIBROSIS Thoracic spinal osteophytes causing focal pulmonary opacities Atelectasis +/- fibrosis (Focal pulmonary fibrosis) Medial basal segment of Rt lower lobe Posterior segment of Left lower lobe

16 Focal lung fibrosis adjacent to osteophytoses in one of the thoracic vertebrae

17 LANGERHANS CELL HISTIOCYTOSIS Multisystem disease Pulmonary involvement seen mostly in young adult smokers -Nodules (Micronodular/ Reticulonodular opacities) -Bizarre shaped cysts -Upper zone predominance -CP angles spared DDx.. Lymphangiomyomatosis

18 Known case of Langerhans Cell Histiocytosis showing upper lobe predominant, bizarre shaped cystic lesions

19 ERDHEIM- CHESTER DISEASE Rare disease Infiltration of mononuclear cells in tissues Lower limb osteosclerosis 50% patients have extra skeletal involvement, 20-30% in lungs PULMONARY INVOLVEMENT Smooth Pleural, Fissural and Interseptal thickening Cystic areas Ground glass opacities

20 SYSTEMIC LUPUS ERYTHEMATOSUS Pleuropulmonary involvement is a common feature of the disease, seen in 50-70% patients. Reduced Diffusion Capacity seen in 88% patients of SLE. CXR appears normal in almost 40% of these patients. PLEURAL INVOLVEMENT Pleuritis Effusions Residual pleural thickening - 70% Must exclude infection as cause NSIP type of lung fibrosis PARENCHYMAL Acute Lupus Pneumonitis. Uncommon but life threatening. Exclude infection, acute pulmonary edema, hemorrhage and infarction. Alveolar Hemorrhage. Pulmonary thromboembolic disease. Pulmonary arterial hypertension. Lymphocytic Interstitial Pneumonia (rare)

21 SECONDARY INVOLVEMENT Infections (conventional or opportunistic. Commonest pulmonary feature in SLE) Atelectasis Diaphragmatic myopathy, Embolic disease, Diaphragmatic splinting from painful pleuritis Congestive Cardiac F/ RF related pulmonary edema Exclude infection + Acute Lupus Pneumonitis Drug (procainamide, hydralazine)/ Oxygen toxicity

22 Lymphocytic interstitial pneumonia (LIP) showing septal thickening and scattered thin walled cysts in a patient of systemic lupus erythematosus

23 ANKYLOSING SPONDYLITIS Upto 15% of the patients have pulmonary involvement. Apical Fibrobullous Disease +/- Mycetoma (tuberculous/ fungal) Ankylosis of costo-vertebral joints

24 COMMONEST PULMONARY MANIFESTATIONS

25 Rheumatoid Arthritis Pleuritis/Pleural thickening Pleural Effusion Interstitial Lung Disease Bronchiectasis Scleroderma Dilated, patulous esophagus UIP type lung fibrosis Pulmonary arterial hypertension Pneumonitits

26 Hypertrophic osteoarthropathy Osteophyte Induced Fibrosis Etiologies Squamous Cell Carcinoma Pulmonary Lymphoma Lung Abscess Focal Pulmonary Fibrosis Adjacent to thoracic spine osteophytes

27 Langerhans Cell Histiocytosis Mid-/ Upper zone micronodular/ reticulonodular opacities / bizarre cysts Sparing of costo phrenic angles Systemic lupus erythematosus Pleuritis Pleural Effusion Interstitial Fibrosis Infections

28 Ankylosing Spondylitis Erdheim- Chester Disease Ankylosis of costovertebral joints Apical fibrobullous disease Smooth pleural, fissural, interlobular septal thickening Upper zone predominance Periaortic soft tissue infiltration

29 CONCLUSIONS Knowledge of the common thoracic manifestations of skeletal disorders and complications related to treatment is essential for optimum management. Overlapping pulmonary features amongst these disorders makes it even more important to be fully aware of these manifestations so the radiologist can collaborate with the clinician and pathologist and play a constructive role in diagnosis and treatment.

30 References 1-Padley SPG, Hansell DM, Flower CDR, et al. (1991) Comparative accuracy of high resolution computed tomography and chest radiography in the diagnosis of chronic diffuse infiltrative lung disease. Clin Radiol 44: Bonelli FS, Hartman TE, Swenson J, et al. (1998) Accuracy of high-resolution CT in diagnosing lung diseases. AJR170: Swensen SJ, Aughenbaugh GL, Myers JL (1997) Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung. Radiology 205: Leung AN, Miller RR, Muller NL. (1993) Parenchymal opacification in chronic infiltrative lung diseases: CT-pathologic correlation. Radiology 188: Masi AT, Rodnan GP, Medsger TA, et al. (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma).arthritis Rheum 23: Padley S, Varma N, Flower CDR. (1990) Case report: tracheobronchomegaly in association with ankylosing spondylitis. Clin Radiol 43: Qian X, Qin J.Oncol Lett Jun;7(6): Otake S, Takahashi M, Ishigaki T. (2002) Focal pulmonary interstitial opacities adjacent to thoracic spine osteophytes. AJR Am J Roentgenol. 179(4):893-6.

31 Pulmonary Manifestations Of Skeletal Disorders U. A. Saeed, MBBS FCPS, J. Nair, MBBS MD, R. Khosla, MD FRCR, K. Sayegh, MD FRCPC, J. Kosiuk, MD FRCPC, J. Taylor, MD FRCPC; Department of Radiology, McGill University Health Centre, Montreal, QC, CANADA. SPECIAL ACKNOWLEDGEMENT: Carl Frédéric Duchatellier, MD

32 Author contact information Dr. Umar Abid Saeed