PANCE Blueprint Pulmonary (10%)

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
  • The etiology of sarcoidosis is unknown although some people appear to have a genetic predisposition to develop the disease, which may be triggered by bacteria, viruses, dust, or chemicals
  • Symptoms are variable - 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 lymphadenopathy 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, zookeeper =  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

"Hypercalcemia in sarcoidosis is due to the uncontrolled synthesis of 1,25-dihydroxyvitamin D3 by macrophages. 1,25-dihydroxyvitamin D3 leads to increased absorption of calcium in the intestine and to increased resorption of calcium in the bone."

Sarcoidosis Smarty PANCE

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.

osmosis Osmosis
Picmonic
Sarcoidosis

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 a routine x-ray.

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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
Question 2
A 32-year-old male with sarcoidosis presents to the clinic complaining of shortness of breath, fatigue, and palpitations for a few months. His in-office EKG reveals QRS prolongation and some premature ventricular beats. A chest radiograph shows cephalization of the pulmonary vessels and cardiomegaly. Which of the following is the most likely diagnosis?
A
Cryptogenic organizing pneumonia
Hint:
Cryptogenic organizing pneumonia is a type of diffuse interstitial lung disease that typically presents with cough, fever, dyspnea, and malaise.
B
Hypertensive encephalopathy
Hint:
Hypertensive encephalopathy manifests as headaches, vomiting, confusion, and neurological symptoms. The patient has none of these.
C
Cardiac sarcoidosis
D
Acute coronary syndrome
Hint:
Acute coronary syndrome is more likely to present with diaphoresis and crushing chest pain.
E
Pneumoconiosis
Hint:
Pneumoconiosis is a fancy word that means the accumulation of dust (e.g., coal, asbestos, etc.) within the lungs. This patient has absolutely nothing in his history that suggests this.
Question 2 Explanation: 
Sarcoidosis is a multisystem granulomatous disorder; it impacts the lungs, eyes, lymph nodes, heart, skin, and nervous system. Cardiac sarcoidosis (CS) is often under-recognized and can occur as a clinical feature of sarcoidosis. Presenting symptoms may include palpitations, syncope, fatigue, & dyspnea. CS happens because granulomas infiltrate the heart, leading to conduction issues, tachyarrhythmias, cardiomyopathies, & heart failure. Holter monitoring and echocardiography are two helpful tests. The gold standard would be a heart biopsy (not routinely done because it is so invasive; cardiac MRI is preferred). Treatment is steroid therapy and anti-arrhythmic drugs.
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