Sarcoidosis -Goya

Question 1

What is sarcoidosis?

Answer 1

a multisystem granulomatous disorder of unknown etiology that is characterized by the presence of noncaseating granulomas in the involved organs

Commonly presents with bilateral hilar lymphadenopathy, pulmonary infiltration & skin lesions.

Symptoms related to Lung, Skin, Eyes, Peripheral nerves, Liver, Kidney, Heart …are possible.

more common in North American blacks (women > men) and North Europen whites

3rd or 4th decade

spontaneous remission in majority of cases

Question 2

What is the pathogenesis of sarcoidosis?

Answer 2

noncaseating granulomas composed of a central core of epithelioid histocytes and multinucleate giant cells

activated T cells and macrophages accumulate at site of inflammation

release chemoattractants and growth factor –> cell proliferation and granuloma formation

progressive granulomatous inflammation leads to injury, dysfunction and destruction of affected organs

fibrosis

Question 3

What is the most common organ system affected by sarcoidosis?

Answer 3

respiratory tract (SOB, nonproductive cough, nonspecific CP, hemoptysis)

present asymptomatically 40% of the time

Question 4

What are some other presenting symptoms in sarcoidosis?

Answer 4

• skin manifestations–> erythema nodosum =raised skin lesions over the shins (Lofgren’s syndrome)
• occular involvement (35% get conjunctivitis)
• arthralgias and arthritis
• neuro: poor prognosis. Cranial nerve palsy, paresthesias, seizures.

Question 5

A 42-year-old female comes to the physician’s office with persistent cough from last 1 year. She had unprotected sex with multiple partners in last 1 year. Four months ago she noticed red bumps on her legs. She also had swelling of her joints. Her HIV test is negative. Her chest x-ray shows bilateral hilar masses. Which of the following is most likely diagnosis?
A. Acute HIV disease
B. Tuberculosis
C. Sarcoidosis
D. Berylliosis

Answer 5

C. Sarcoidosis

Question 6

What chest x-ray findings are associated with the different stages of sarcoidosis?

Answer 6

stage 1:

• Bilateral hilar lymphadenopathy with or without paratracheal adenopathy
• Normal lung parenchyma
• 50% present with this as asymptomatic

Stage 2

• bilateral hilar adenopathy with lung involvement
• 25% present in this stage.
• symptomatic (1/3 will progress to fibrosis)

stage 3: (lung only)

• significant fibrosis without bilateral hilar adenopathy
• 15% present with this

Stage 4:

• pulmonary fibrosis with honeycombing
• worse in upper lobes

Question 7

What lab abnormalities are seen in sarcoidosis? Are these specific?

Answer 7

elevated ACE in 50-80% of pts

hypercalcemia/hypercalcuria

elevated LFT

elevated creatinine

*not specific to sarcoidosis

Question 8

What is the diagnostic criteria for sarcoidosis?

Answer 8

appropriate clinical picture, histologic findings & exclusion of other potential diseases capable of producing a similar picture

Fiberoptic bronchoscopy is the most common method for obtaining a diagnosis

Biopsy is 50-60% in patients without parenchymal disease

90-95% in patients with parenchymal disease

Question 9

What are the indications for steroid use in sarcoidosis? Do pts with stage 1 disease an normal lung function require treatment with steroids? Stage 2-3?

Answer 9

Progressive pulmonary impairment or respiratory symptoms

Ocular involvement

Myocardial involvement

CNS sarcoidosis

Persistent hypercalcemia/hypercalicuria with renal insufficiency

*Patients with stage 1 disease with or without erythema nodosum & with normal lung function (VC, DLCO) do not require treatment with corticosteroids

stage 2-3 with symptoms and impaired lung functions should be treated with steroids

Question 10

What features suggest poor prognosis in sarcoidosis?

Answer 10

Insidious onset with cutaneous, eye or bone involvement

CXR stages 2 or 3

Alveolar or fibrotic changes on CXR

Abnormal PFTs

Progression of organ involvement beyond 1 year

Question 11

A 49-year-old African American woman comes to the physician because of shortness of breath, cough, and chest pain. Chest x-ray shows hilar lymphadenopathy and biopsy of the lymph node is consistent with a diagnosis of sarcoidosis. Which of the following histologic findings in the biopsy specimen best confirms the diagnosis? 
A. Aschoff bodies
B. Calcifications
C. Caseous necrosis
D. Epitheloid histiocytes

Answer 11

D. Epitheloid histiocytes