PANCE Blueprint Pulmonary (12%)

Sarcoidosis

Patient will present as → a 30-year-old African American female with a cough, fever, and generalized body aches. You order a CXR which shows bilateral hilar adenopathy

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Sarcoidosis is a systemic granulomatous disease that is characterized by noncaseating granulomas that may affect multiple organ systems

  • The condition occurs mainly in persons ages 20 to 40 years and is most common in Northern Europeans and African Americans
  • Symptoms are variable and the etiology is unknown. Fever, weight loss, arthralgias, and erythema nodosum (more commonly seen in Europeans) are the usual initial presenting symptoms. Cough and dyspnea may be minimal or absent.

MEDIASTINAL LYMPHADENOPATHY seen on chest radiograph is the hallmark finding in 90% of cases

"If you see hilar lymphadenopthy it is right to think Sarcoidosis, but not always. Here is a rough guide to approaching hilar adenopathy: 

  • Young female = Sarcoidosis
  • Young kid with a fever, from Ohio, zoo keeper =  histoplasmosis 
  • Old guy in his 60's works on ceramics = Berylliosis"

Other manifestations include hepatic granulomas, granulomatous uveitis, polyarthritis, cardiac symptoms (including angina, CHF, and conduction abnormalities), cranial-nerve palsies, and diabetes insipidus

Serum blood tests will show hypercalcemia and ACE levels 4 x normal

  • ESR is often elevated
  • Laboratory findings include leukopenia, hypercalcemia, hypercalciuria, and hypergammaglobulinemia (particularly in African American patients)
  • Other abnormalities include elevated uric acid (not usually associated with gout), elevated alkaline phosphatase, elevated gamma glutamyl transpeptidase, elevated levels of ACE, and pulmonary function tests showing restriction and impaired diffusing capacity
  • Diagnosis can be made with a biopsy of peripheral lesions or fiber-optic bronchoscopy for central pulmonary lesions. Whole-body gallium scans can be used to show useful sites for biopsy and, in some cases, to follow disease progression

Serial pulmonary function tests are important for assessing disease progression and guiding treatment

Bilateral hilar adenopathy

Bilateral hilar adenopathy in a patient with sarcoidosis.

Treatment for symptomatic patients consists of CORTICOSTEROIDS, methotrexate, and other immunosuppressive medications if steroid therapy is not helpful

  • 90% of cases are responsive to corticosteroids and can be controlled with a modest maintenance dose
  • Ace Inhibitors for periodic hypertension

The prognosis depends on the severity of the disease. Spontaneous improvement is common; however, significant disability can occur with multiorgan involvement

  • Pulmonary fibrosis is the leading cause of death.

IM_MED_SarcoidosisCharacteristics_v1.5_ Sarcoidosis is an autoimmune disease characterized by noncaseating granulomas throughout the body. It is characterized by lab findings such as increased levels of ACE, and asteroid bodies. Due to vitamin D activation, hypercalcemia is present. This disease is often asymptomatic, and is diagnosed after finding bilateral hilar lymphadenopathy on routine x-ray.

View Sarcoidosis Picmonic

 

Question 1
A 52-year-old man is seen for fevers and weight loss. A chest radiograph shows mediastinal lymphadenopathy. Laboratory findings show hypercalcemia, elevated alkaline phosphatase, and an elevated level of ACE. The most likely diagnosis is
A
Small cell carcinoma of the lung
B
Pulmonary tuberculosis
C
Sarcoidosis
D
Histoplasmosis
E
Asbestosis
Question 1 Explanation: 
Sarcoidosis is a systemic granulomatous disease that is characterized by noncaseating granulomas that may affect multiple organ systems. The condition occurs mainly in persons ages 20 to 40 years and is most common in Northern Europeans and African Americans. Symptoms are variable and the etiology is unknown. Fever, weight loss, arthralgias, and erythema nodosum (more commonly seen in Europeans) are the usual initial presenting symptoms. Cough and dyspnea may be minimal or absent. Other manifestations include mediastinal lymphadenopathy seen on chest radiograph (hallmark finding in 90% of cases), hepatic granulomas, granulomatous uveitis, polyarthritis, cardiac symptoms (including angina, CHF, and conduction abnormalities), cranial-nerve palsies, and diabetes insipidus. Laboratory findings include leukopenia, hypercalcemia, hypercalciuria, and hypergammaglobulinemia (particularly in African American patients). Other abnormalities include elevated uric acid (not usually associated with gout), elevated alkaline phosphatase, elevated gamma glutamyl transpeptidase, elevated levels of ACE, and pulmonary function tests showing restriction and impaired diffusing capacity. Diagnosis can be made with biopsy of peripheral lesions or fiber-optic bronchoscopy for central pulmonary lesions. Whole-body gallium scans can be used to show useful sites for biopsy and, in some cases, to follow disease progression. Serial pulmonary function tests are important for assessing disease progression and guiding treatment. The prognosis depends on the severity of the disease. Spontaneous improvement is common; however, significant disability can occur with multiorgan involvement. Pulmonary fibrosis is the leading cause of death. Treatment for symptomatic patients consists of corticosteroids, methotrexate, and other immunosuppressive medications if steroid therapy is not helpful. Source: Bratton's Family Medicine Board Review
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