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

I ’m coming t o get YOU!

DISCLOSURES

NO RELEVANT DISCLOSURES

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

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.

SKELETAL DISORDERS UNDER DISCUSSION

• Rheumatoid Arthritis

• Scleroderma

• Hypertrophic Osteoarthropathy

• Osteopyte Induced Fibrosis

• Langerhans Cell Histiocytosis

• Erdheim- Chester Disease

• Systemic lupus erythematosis

• Ankylosing spondylitis

RHEUMATOID ARTHRITIS

• Following infections, pleuropulmonary involvement is the second most common cause of death in patients with Rheumatoid Arthritis, after infections.

• Significant prognostic value

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

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

SCLERODERMA

Mediastinum

-Dilated, patulous esophagus

-Rare – Esophageal cancer

Parenchymal involvement

- NSIP type of ILD

-Pulmonary hypertension

- Pnuemonitis

aspiration

infection

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

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

HYPERTROPHIC OSTEOARTHROPATHY

• Bronchogenic Carcinoma

• Pulmonary Lymphoma

• Lung Abscess

• Bronchiectasis

• Pulmonary Metastases (especially osteosarcoma)

• Pleural Fibroma

• Mesothelioma

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

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

Focal lung fibrosis adjacent to osteophytosesin one of the thoracic vertebrae

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

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

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

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

PARENCHYMAL

• NSIP type of lung fibrosis

• 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)

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

Lymphocytic interstitial pneumonia (LIP) showing septalthickening and scattered thin walled cysts in a patient

of systemic lupus erythematosus

ANKYLOSING SPONDYLITIS

•Upto 15% of the patients have pulmonary involvement.

•Apical Fibrobullous Disease +/- Mycetoma (tuberculous/ fungal)

•Ankylosis of costo-vertebral joints

COMMONEST PULMONARY MANIFESTATIONS

Rheumatoid Arthritis

• Pleuritis/Pleural thickening

• Pleural Effusion

• Interstitial Lung Disease

• Bronchiectasis

Scleroderma

•Dilated , patulous esophagus

•UIP type lung fibrosis

•Pulmonary arterial hypertension

•Pneumonitits

Hypertrophic osteoarthropathy

Etiologies

• Squamous Cell Carcinoma

• Pulmonary Lymphoma

• Lung Abscess

Osteophyte Induced Fibrosis

• Focal Pulmonary Fibrosis

• Adjacent to thoracic spine osteophytes

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

Ankylosing Spondylitis

• Ankylosis of costovertebral joints

• Apical fibrobullous disease

Erdheim- Chester Disease

• Smooth pleural, fissural, interlobular septal thickening

• Upper zone predominance

• Periaortic soft tissue infiltration

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.

References

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2- Bonelli FS, Hartman TE, Swenson J, et al. (1998) Accuracy of high-resolution CT in diagnosing lung diseases. AJR170:1507–1512.

3- Swensen SJ, Aughenbaugh GL, Myers JL (1997) Diffuse lung disease: diagnostic accuracy of CT in patients undergoing surgical biopsy of the lung. Radiology 205:229–234.

4-Leung AN, Miller RR, Muller NL. (1993) Parenchymal opacification in chronic infiltrative lung diseases: CT-pathologic correlation. Radiology 188:209–214.

5-Masi AT, Rodnan GP, Medsger TA, et al. (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma).Arthritis Rheum 23:581–590.

6-Padley S, Varma N, Flower CDR. (1990) Case report: tracheobronchomegaly in association with ankylosing spondylitis. ClinRadiol 43:139–141.

7-Qian X, Qin J.Oncol Lett. 2014 Jun;7(6):2079-2082.

8-Otake S, Takahashi M, Ishigaki T. (2002) Focal pulmonary interstitial opacities adjacent to thoracic spine osteophytes. AJR Am J Roentgenol. 179(4):893-6.

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

Author contact information

Dr. Umar Abid Saeed

umarabid@gmail.com