Updated: 3/23/2020

Sarcoidosis

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Snapshot
  • potatonodeA previously healthy 30-year-old African American woman has fatigue, arthralgia, and a nodular rash over the trunk and upper extremities for three weeks. There are twelve 3-8 mm, pale, indurated plaques over the chest, back, and upper extremities. The liver is palpable 2 cm below the right costal margin with a percussion span of 14 cm, and the spleen tip is palpable 3 cm below the left costal margin. There is no pain or limitation of any of the joints. A chest radiograph shows bilateral lymphadenopathy.
Introduction
  • Idiopathic condition characterized by granulmomatous inflammation of multiple organs
    • lungs most commonly involved organ
    • commonly causes restrictive lung disease 
      • may also cause obstructive or mixed pattern
    • mediated by CD4 TH cells
      • ↑ IFN-gamma 
    • granulomas can affect all organ systems
      • liver/spleen
      • bone
      • heart
  • Epidemiology
    • most common in black females
    • smoking does not ↑ risk
    • presents most commonly in 3rd or 4th decade
  • Associated conditions
    • diabetes insipidus (granulomatous infiltration of posterior pituitary)
Presentation
  • Symptoms
    • may be asymptomatic
      • 50% as incidental chest radiograph findings
    • cough
    • fever, malaise
    • arthritis
      • symptoms mainly in the ankle and legs
    • can be GRUELING
      • Granulomas
      • Rheumatoid arthritis
      • Uveitis
      • Erythema nodosum
      • Lymphadenopahy
      • Interstitial fibrosis
      • Negative TB
      • Gammaglobulinemia
  • Physical exam
    • skin lesions
      • nodular granulomatous lesions
      • facial rash
      • erthyma nodosum 
        • painful nodules
        • also seen in rheumatic fever
    • eye lesions
      • uveitis 
        • ↓ vision and glaucoma
    • enlarged salivary and lacrimal glands
Evaluation
  • Diagnosis is clinical and often one of exclusion
  • Chest radiograph
    • bilateral hilar lymphadenopathy
    • lungs involved in 90% of the cases
  • Labs
    • ↑ serum ACE (not uniformly) 
    • hypercalcemia 
      • ↑ 1-α-hydroxylase → hypervitaminosis D
        • can cause hypercalemia and renal failure chronically
      • commonly tested, less commonly seen (only about 11%)
    • serum protein electrophoresis (SPE) shows polyclonal gammopathy
    • lack of response to CD4 TH skin tests (like PPD) due to ↑ lung activity and  systemic activity
  • Pulmonary function tests
    • restrictive pattern is common (normal FEV1/FVC with normal TLC)
      • however, obstructive or mixed pattern may also be seen
  • Bronchoalveolar lavage
    • high CD4:CD8 T-cell ratio 
      • due to CD4 infiltrate into the interstitium as well as intralveolar space
      • contrasted to the low ratio in hypersensitivity pneumonitis and HIV
  • Biopsy 
    • noncaseating granulomas with multinucleated giant cells   
      • very suggestive of sarcoidosis 
    • laminated calcium concretions present (cause of density on CXR)
Treatment
  • Conservative
    • observation fine in most cases 
      • majority experience remission within two years without treatment
  • Pharmacologic
    • topical preferred when possible (skin, eyes, nasal, airway, etc)
    • corticosteroids
    • indications
      • extra-pulmonary manifestations: cardiac, neurologic, or uveitis
      • symptomatic hepatosplenomegaly
      • significant hypercalcemia
      • only if refractory to observations
Prognosis
  • Relapsing/remitting course with 50% resolving spontaneously
  • Progression
    • Stage I - bilateral hilar lymphadenopathy
    • Stage II - bilateral hilar lymphadenopathy + upper lobe infiltrates
    • Stage III - lung infiltrates only
    • Stage IV - lung fibrosis
 

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(M1.PL.13.42) A 33-year-old African-American female presents to her physician with complaints of a persistent, dry cough. She states that the cough has gone on for some time now. Three weeks ago, during her last general checkup, she was started on lisinopril and metformin for concerns regarding an elevated blood pressure and fasting blood glucose. Past medical history is notable for eczema, asthma, and seasonal allergies. At this visit the patient has other non-specific complaints such as fatigue and joint pain as well as a burning sensation in her sternum when she eats large meals. Her physical exam is only notable for painful bumps on her lower extremities (figure A) which the patient attributes to "bumping her shins," during exercise, and an obese habitus. Which of the following is most likely true for this patient's underlying diagnosis? Tested Concept

QID: 100845
FIGURES:
1

Serum levels of bradykinin will be elevated

23%

(8/35)

2

Loratadine would best treat her chief complaint

0%

(0/35)

3

Beta agonists would relieve this patients symptoms

0%

(0/35)

4

Non-caseating granulomas are found on biopsy of mediastinal lymph nodes

71%

(25/35)

5

Omeprazole is an appropriate next step in management

3%

(1/35)

M 2 E

Select Answer to see Preferred Response

(M1.PL.13.115) A 45-year-old female presents with fatigue, fever, and a nodular rash over her trunk and extremities that she's had for 3 weeks. CXR was performed (Figure A) and subsequently the patient underwent transbronchial lung biopsy (Figure B). Which abnormality would be most likely in an analysis of this patient's serum? Tested Concept

QID: 100918
FIGURES:
1

Hypercalcemia

77%

(27/35)

2

Decreased ACE

9%

(3/35)

3

Hypovitaminosis D

3%

(1/35)

4

Increased ADH

0%

(0/35)

5

Increased Parathyroid Hormone Related Peptide

9%

(3/35)

M 2 E

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(M1.PL.13.34) A 34-year-old African American female presents complaining of worsening dyspnea and hemoptysis. Serum ACE (angiotensin converting enzyme) levels are elevated. A chest x-ray shown in Figure A suggests the likely diagnosis. Which of the following findings is consistent with this diagnosis? Tested Concept

QID: 100837
FIGURES:
1

Type I hypersensitivity reaction

6%

(2/34)

2

Caseating granulomas

3%

(1/34)

3

Non-caseating granulomas

85%

(29/34)

4

Ectopic production of ADH

6%

(2/34)

5

Charcot-Leyden crystals

0%

(0/34)

M 2 D

Select Answer to see Preferred Response

(M1.PL.13.142) A 38-year-old African American female presents to her primary care physician with uveitis, cough, and arthralgias in her ankles and legs. Bloodwork reveals elevated angiotensin converting enzyme levels, and skin PPD testing shows no observable induration after 48 hours. The patient demonstrates reduced FEV1 and FVC upon spirometry. FEV1/FVC is 85%. Which of the following would you expect to see upon chest X-ray: Tested Concept

QID: 100945
1

Enlarged hilar lymph nodes

86%

(42/49)

2

Kerley B Lines

2%

(1/49)

3

Bilateral diaphragmatic elevation.

4%

(2/49)

4

Pleural effusion

0%

(0/49)

5

Fluid in alveolar walls

6%

(3/49)

M 2 D

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(M1.PL.13.30) A 31 year-old African-American female presents with a painful shin nodules, uveitis, and calcified hilar lymph nodes. A transbronchial biopsy of the lung would most likely show which of the following histologies? Tested Concept

QID: 100833
1

Inflammation, fibrosis and cyst formation that is most prominent in subpleural regions

4%

(5/127)

2

Silica particles (birefringent) surrounded by collagen

2%

(2/127)

3

Golden-brown fusiform rods

3%

(4/127)

4

Patchy interstitial lymphoid infiltrate into walls of alveolar units

3%

(4/127)

5

Non-caseating granulomas

87%

(111/127)

M 2 D

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