pathophysiology, diagnosis, and treatment amitabh k...

arcoidosis

Amitabh

K. Bharadwaj, Harvard Medical School, Year-IVGillian Lieberman, MD

Pathophysiology, Diagnosis, and Treatment

September 2000Amitabh K. BharadwajGillian B. Lieberman, MD

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Fever of Unknown Etiology

• M. S. is a 52 yo white F with a history of scleroderma and cholelithiasis who presented with dry cough, shaking chills, and fevers to 102 F despite being put on amp/gent/flagyl

Amitabh K. BharadwajGillian B. Lieberman, MD

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Chest film• There were no

abnormalities identified

BIDMC


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In view of her significant presentation, a chest and abdominal CT were ordered.

• A chest CT showed mediastinal lymphadenopathy, and an interstitial reticulo nodular pattern predominantly in the upper lobes.

Mediastinal adenopathy

Interstitial markings Nodules

Chest CT


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Ddx Upper Lobe Interstitial Process

• Silicosis• Sarcoid• Atypical PCP• Hypersensitivity Pneumonitis• Ankylosing spondylitis• Langerhans cell histiocytosis


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Patient’s Abdominal CT

Abdominal CT showed: • 1.8 cm gallstone

without signs of cholecystitis.

• Multiple low- attenuation splenic lesions

BIDMC Low-attenuated splenic lesions8mm hepatic cyst(incidental finding)


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Lung and spleen findings suggest Sarcoidosis. Splenic and lung

biopsies were obtained and showed non caseating

granulomata confirming the diagnosis of Sarcoidosis


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The patient underwent a laporoscopic cholecystectomy

and splenectomy. Her fever and night sweats abated and she was

discharged without further treatment.


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Follow up Chest X-ray• 4 mo later she came

back for outpatient f/u and her CXR showed she had developed hilar adenopathy

BIDMC

Bilateral hilar adenopathy


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Repeat Chest CT• Mediastinal windows

showed:Hilar and mediastinal lymph node enlargement

Mediastinal(subcarinal)adenopathy

Hilar adenopathy

BIDMC


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Repeat Chest CTLung windows showed:Numerous small sub-cm lungnodules bilaterally

Lung nodules

BIDMC


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Chest findings are compatible with progression of stage 11

sarcoidosis


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Sarcoidosis• F > M, 20-40 yo• United States: black > white 10:1 – 17:1• Europe: disease affects mostly whites• Chronic multisystem disorder of unknown etiology • Noncaseating granulomas in affected organs:

T lymphocytes and macrophages • Most frequently symptomatic organs:

– Lung 90%– Lymph nodes 75-90%– Skin/eye/liver 25% each– Bone marrow/spleen 15-40%– CNS/MS/heart 5% each


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Disease Presentation• Most patients have some respiratory symptoms• Subacute sarcoidosis: 20-40%

– Fever, malaise, anorexia, or weight loss– +/- Dry cough, dyspnea, retrosternal chest

discomfort– Often self-limited

• Chronic sarcoidosis: 40-70%– Dry cough, dyspnea, or retrosternal chest

discomfort– +/- Fever, malaise, anorexia, weight loss– May progress to permanent lung and secondary

organ damage


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Diagnosis

Required for diagnosis:• History / Physical exam• Negative blood tests and positive

pulmonary function tests• CXR indicative of sarcoidosis• Biopsy evidence of noncaseating

granulomatous process– lung parenchyma (usual site, via bronchoscopy)– hilar nodes, skin, conjunctiva, lip


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Presentation of Pulmonary Sarcoid on Chest XRay


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CXR: Sarcoid Stage I• In stage I disease, there is hilar (± mediastinal)

adenopathy without interstitial infiltrate

Hilar adenopathy

From: Teaching file of Dr. Philip Boiselle; Beth Israel Deaconess Medical Center, Boston MA


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Differential for Hilar Adenopathy

• Tb - usually unilateral• Sarcoid - usually bilateral, symmetric• Fungal infection• Metastatic disease - especially renal, thyroid• Lymphoma/leukemia• Benign lymph node hyperplasia

(Castleman’s disease)


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CXR: Sarcoid Stage II

• In stage II disease, hilar adenopathy is accompanied by interstitial infiltrate, which tends to be more prominent at the apices as in this patient

Apicalinfiltrate

Hilar adenopathy



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CXR: Sarcoid Stage III• In stage III disease, hilar

LAN has disappeared and diffuse interstitial infiltrates remain

• Differential includes: CHF, lymphangitic spread of CA, infection (viral, mycoplasma), sarcoid, pneumoconiosis, collagen vascular disease

Diffuse interstitialinfiltrates



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CXR: Sarcoid Stage IV

• Presents with fibrosis as in this radiograph

From: Loyola University - www.lumen.luc.edu/lumen/MedEd/Radio/Sarc/introxry.htm

Fibrotic bands


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CXR: Sarcoid Stage IV• Sometimes can cause cystic spaces on CXR, aka

“cystic sarcoid”

Cystic sarcoid



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Differential for fibrosis

• Collagen vascular disease (RA, scleroderma)• Sarcoid stage IV• Silicosis• Asbestosis• Hypersensitivity pneumonitis• Idiopathic fibrosis• Drug/radiation toxicity


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Pulmonary Sarcoid on Chest CT


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Sarcoid on CT

• 1. Hilar and mediastinal adenopathy

Symmetric hilar adenopathyBulky mediastinaladenopathy (precarinal)



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Sarcoid on CT• 2. Interstitial markings and perilymphatic nodules

Perilymphatic nodules

Interstitial markings



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Sarcoid on CT• 3. Fibrosis (end-stage)• “Honey combing”

From: Webb WR, Brandt WE, Helms CA. 1998. Fundamentals of Body CT. W.B. Saunders Company.


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HRCT Findings in Sarcoid

• 1. Perilymphatic nodules, 1-10 mm, often subpleural, may calcify

• 2. Patchy, often asymmetric, distribution• 3. Upper lobe predominance • 4. Hilar and mediastinal LAN (not always;

may calcify)• 5. Ground-glass opacity (uncommon),

indicating the presence of small granulomas


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Treatment of Sarcoidosis• Treat early - Permanent organ derangements are

not responsive to glucocorticoids• Oral prednisone• Repeat treatment protocol for reactivations • Other considerations

– inhaled glucocorticoids not efficacious– mild ocular disease: local therapy– uveitis: systemic therapy


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References

• BIDMC, BIDMC Patient Care Radiology Files. 2000. Beth Israel Deaconess Medical Center, Boston MA. SLIDE#3, 4, 5, 7, 8, 9

• Boiselle, P. 2000. Personal teaching file, Beth Israel Deaconess Medical Center, Boston MA. SLIDE#13, 15, 16, 18, 21, 22

• Freiman DG. Pathology of Sarcoidosis. 1985. Semin Roentgenol 20:356-375. • Isselbacher K et al. 1994. Harrison’s Principles of Internal Medicine. McGraw-

Hill Publishing.• Loyola University Medical Education Network. 2000. Website address:

www.lumen.luc.edu/lumen/MedEd/Radio/Sarc/introxry.htm. SLIDE#17 • Miller BH, Rosado-de-Christenson ML, McAdams HP, Fishback NF. 1995.

RadioGraphics: 15:421-437. • Sanguinetti CM, Montroni M, Balbi B, Prete M, Gasparini S, Ross FA. 1987. Does

activity of pulmonary sarcoidosis depend on disease duration: a correlation between bronchoalveolar lavage, scintigraph, radiologic, and physiologic parameters and time of onset of disease. Sarcoidosis 4:18-24.

• Webb WR, Brandt WE, Helms CA. 1998. Fundamentals of Body CT. W.B. Saunders Company. SLIDE#19, 20, 23


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Acknowledgements

Phillip Boiselle for his generous contribution of cases.Beverlee Turner for her efforts in making the final touches.


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The End


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