10-9 DSA - Sarcoidosis by Kinder

Question 1

What is sarcoidosis?

What organs can be affected?

What is present in a vast majority of cases?

Answer 1

1. Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized pathologically by the non-caseasting granuloma.
2. The disease can affect almost any organ in the body.
3. Over 90% of patients present with mediastinal and hilar lymph node involvement or parenchymal lung disease.

Question 2

Who does sarcoidosis most frequently affect?

Answer 2

Sarcoidosis can occur at any age, but most commonly between 40-60 years.

In the U.S. the incidence is 3.5 times higher in black Americans compared to white Americans.

Women have a higher incidence than men.

Question 3

How does sarcoidosis frequently present?

Answer 3

Pulmonary involvement is frequent, but not necessary for the diagnosis.

Sarcoidosis may initially present with an abnormal x-ray as part of a screening examination with enlarged mediastinal and hilar lymph nodes.

Up to 50% of patients have no symptoms at the time of initial diagnosis

Question 4

What is Lofgren’s syndrome?

Answer 4

Lofgren’s syndrome is an acute manifestation of the disease consisting of erythema nodosum, arthritis, and bilateral hilar lymphadenopathy.

Erythema nodosum is more common in women and arthritis is more common in men.

Lofgren’s syndrome has a favorable prognosis.

Question 5

What are the general symptoms consistent with sarcoidosis?

Answer 5

fatigue, fever, night sweats, and weight loss

Question 6

What are the pulmonary signs and symptoms consistent with sarcoidosis?

Answer 6

cough, dyspnea with exertion, wheeze, chest discomfort – 50% of patients have only respiratory symptoms

signs:

rales, expiratory wheezing, can also be normal

Question 7

What are the skin signs and symptoms consistent with sarcoidosis?

Answer 7

rash, macules, papules, nodules, hyperpigmentation or hypopigmentation, erythema nodosum

signs:

infiltration of old scars and tattoos, Maculopapular lesions of face, neck , upper back, and extremities, Lupus pernio of nose, cheeks, ears, and lips, Erythema nodosum

Question 8

What are the ocular signs and symptoms consistent with sarcoidosis?

Answer 8

gritty or dry eyes, pain, redness, and or blurred vision

signs:

uveitis, iritis, scleral plaques, enlarged lacrimal glands

Question 9

What are the cardiac signs and symptoms consistent with sarcoidosis?

Answer 9

arrhythmia, palpitations, near-syncope, syncope, lower extremity edema

signs:

usually normal, irregular rhythm

Question 10

What are the nervous system signs and symptoms consistent with sarcoidosis? 7

Answer 10

1. headaches,
2. blurred vision,
3. numbness,
4. weakness,
5. seizures
6. cranial nerves II, VII, and VIII neuropathies,
7. gait instability,

Question 11

What are the GI system signs and symptoms consistent with sarcoidosis?

Answer 11

epigastric pain, early satiety, RUQ pain, jaundice

heptatomegaly, splenomegaly, jaundice

Question 12

What are the MSK system signs and symptoms consistent with sarcoidosis?

Answer 12

swelling and joint stiffness, joint pain, muscle pain

Signs:

arthralgias, arthritis

Question 13

What are the upper respiratory tract signs and symptoms consistent with sarcoidosis?

Answer 13

nasal congestion, sinus pressure, nasal discharge

(symptoms only)

Question 14

What are the lymphatic signs and symptoms consistent with sarcoidosis?

Answer 14

signs only:

common sites of palpable adeonpathy include cervical, supraclavicular, axillae, and inguinal regions

Question 15

How many patients with sarcoidosis have pulmonary involvement?

Answer 15

Over 90% of patients present with pulmonary involvement.

Mediastinal and hilar lymphadenopathy and/or pulmonary parenchymal disease.

Question 16

How many patients with sarcoidosis have skin involvement? What do you expect to see?

Answer 16

Skin involvement in 25-35% of patients.

Lesions have a predilection for scars and tattoos.

Erythema nodosum occurs in 10% of patients. Erythema nodosum is a raised, red, hot, tender subcutaneous nodule most commonly on the shins. Biospy of erythema nodosum is not useful since these are not sarcoid granulomas.

Lupus pernio affects the nose, cheeks, and ears.

Question 17

How many patients with sarcoidosis have cardiac involvement? What pathologies do you expect to see?

Answer 17

Cardiac granulomas are found in 25% of patients at autopsy.

Cardiac involvement is only suspected in 5% of patients.

Cardiac manifestations can include atrioventricular block, ventricular arrhythmias, left ventricular dysfunction, and sudden death.

Question 18

How many patients with sarcoidosis have neurologic involvement? What pathologies do you expect to see?

Answer 18

Nervous system granulomas in 25% of sarcoidosis patients at autopsy.

10% have neurologic symptoms.

In patients with neurolosarcoidosis, symptoms may precede the diagnosis of sarcoidosis in up to 75% of patients.

Granulomas can occur in the base of the brain, hypothalamus, and pituitary gland.

The most common cranial nerves affected are VII, II, and VIII.

Myelopathy and peripheral neuropathy can occur.

Question 19

How many patients with sarcoidosis have liver and spleen involvement? What pathologies do you expect to see?

Answer 19

Fever, weight loss, and jaundice are present in less than 5% with liver involvement.

20% of patients will have hepatomegaly.

5-15 % of patients will have splenomegaly.

Question 20

How many patients with sarcoidosis have bone and joint involvement? What joints are most commonly involved?

Answer 20

Most patients have arthralgias

35% develop arthritis.

Most common joints affected are the ankles and knees.

Question 21

How many patients with sarcoidosis have calcium metabolism involvement? What pathologies do you expect to see?

Answer 21

Disorders of vitamin D occurs in 50% of patients

may result in nephrolithiasis, nephrocalcinosis, and hypercalcuria.

10-14% of chronic sarcoidosis patients have at least one symptomatic renal stone.

Question 22

How is sarcoidosis dx’ed?

Answer 22

made on clinical, radiographic, and histologic evidence of noncaseating granulomas in one or more organs.

Question 23

What is pathognomonic for sarcoidosis?

Answer 23

Lofgren’s syndrome

• may not require biopsy confirmation.

Question 24

Where should a biopsy for Dx be taken?

Answer 24

Biopsy should be taken from the safest site such as:

skin,

peripheral lymph nodes,

lacrimal glands, or

conjunctiva

Question 25

In addition to biopsy, what is another technique that is helpful for dx'ing sarcoidosis?

Answer 25

Bronchoscopy with transbronchial lung biopsy has a diagnostic yield of at least 85%.

Question 26

What will bronchoalveolar lavage reveal with sarcoidosis?

Answer 26

Bronchoalveolar lavage may be remarkable for lymphocytosis and an elevated CD4/CD8 ratio, however this is not diagnostic

Question 27

What is elevated in sarcoidosis patients?

Answer 27

Sarcoid granulomas produce ACE. ACE levels are elevated in 60% of patients, but this is nonspecific.

Question 28

What are some differential dx's for sarcoidosis?

Answer 28

Infection: Mycobacteria, Fungi, Spriochetes, Bacteria, Parasites Neoplasia: Lymphoma, Carcinoma Extrinsic allergic alveolitis: Animal protein(bird fancier’s lung), Fungi(farmer’s lung) Chemical: silica, beryllium Immunologic: Crohn’s disease, primary biliary cirrhosis, vasculitis(Wegener’s polyangiitis)

Question 29

What would you expect to see on CXR for the different stages of sarcoidosis?

Answer 29

Stage 0: Normal Stage 1: Bilateral hilar adenopathy Stage 2: Bilateral hilar adenopathy and parenchymal infiltrates Stage 3: Parenchymal infiltrates without lymphadenopathy Stage 4: Advanced parenchymal disease with fibrosis

Question 30

What testing would you order for Dx of sarcoidosis, in addition to biopsy and bronchoscopy?

Answer 30

Chest X-ray Staging PFT : Restrictive ventilatory defect, concurrent obstruction, decreased DLCO Opthalmology Exam EKG Consider Cardiac Event Monitoring and Echocardiography CBC CMP to assess liver, renal function, and hypercalcemia 24 hour urine for calcium for hypercalciruria Serum ACE level not sensitive or specific enough Biopsy of involved organ

Question 31

When should you treat sarcoidosis?

Answer 31

Should only be initiated for symptomatic patients.

Question 32

How should you treat sarcoidosis?

Answer 32

Prednisone 20-40 mg daily for 3 months. Monitor for improvement in FVC. If improved wean to 10-15 mg daily for 6-9 months then taper off. Cytotoxic and immunosuppressives are used for those not responding to prednisone.

Question 33

What is the prognosis for sarcoidosis and Lofgren's syndrome?

Answer 33

Spontaneous remission in 50% of patients at 3 years. Lofgren’s with best prognosis. 2/3 remission after 10 years. 1/3 with unrelenting disease. Less than 5% die from sarcoidosis.