RHS12 - Pulmonary Pathology 6

Question 1

What is a diffuse alveolar hemorrhage syndrome? List the ones we need to know.

Answer 1

Diffuse alveolar hemorrhage syndromes are vascular diseases that are the result of a primary immune reaction and manifest as: hemoptysis, anemia, and diffuse pulmonary infiltrates

• Goodpasture Syndrome
• Idiopathic Hemosiderosis
• Wegener Granulomatosis

Question 2

What is Goodpasture Syndrome? Give its pathogenesis and the primary disorders it causes.

Answer 2

An autoimmune disease where the body develops antibodies against the α3 chain of Type IV collagen. This only affects the glomerular and alveolar basement membranes, causing a linear deposition of IgG along the membrane.

This leads to proliferative and rapidly progressive glomerulonephritis as well as hemorrhagic interstitial pneumonitis.

Question 3

What are the clinical features of Goodpasture Syndrome?

Answer 3

• Glomerulonephritis Sx - hematuria, proteinuria, RBC casts, and eventual renal failure.
• Hemorrhagic Interstitial Pneumonitis - SOB and hemoptysis

Question 4

Discribe the gross and histological morphology of Goodpasture Syndrome.

Answer 4

• Gross - lungs will be heavy with reddish-brown consolidation
• Histological - fresh hemorrhage is seen as RBCs in the alveoli. Old hemorrhage is seen as hemosiderrin (the iron taken from the RBCs) laden macrophages in the alveoli. (refer to image)

Question 5

How is Goodpasture Syndrome treated?

Answer 5

• Plasmapheresis
• Immunosuppressive therapy
• Renal transplant required in severe cases

Question 6

What is Wegener Granulomatosis?

Answer 6

• AKA Pulmonary angiitis (swelling of pulmonary vessel walls) with grnaulomatosis (granuloma formation).
• Autoimmune disease where the body forms anti-neutrophil cytoplasmic antibodies (aka - C-ANCA or PR3-ANCA) leading to the formation of
  
  • Granulomas in the URT and/or LRT
  • Vasculitis of small to medium sized vessels
  • Glomerulonephritis

Question 7

What are the clinical features of Wegener Granulomatosis?

Answer 7

• More common in middle aged and elderly males
• Glomerulonephritis Sx - hematuria, proteinuria, RBC casts, eventual renal failure
• Pneumonitis with nodules and cavitary lesions
• Chronic sinusitis
• Mucosal ulcerations of nasopharynx
• Rashes, myalgias, fever

Question 8

Describe the histopathology of Wegener Granulomatosis.

Answer 8

• Loosely formed areas of necrosis
• Loosely formed granulomas with free giant cells
• Vaculitis presenting as either capillaritis or necrotizing

Question 9

How is Wegener Granulomatosis treated?

Answer 9

With steroids, cyclophosphamide, anti-TNF, and/or Rituximab

Question 10

What is Idiopathic Pulmonary Hemosiderosis? Which demographic does it tend to affect?

Answer 10

• A diagnosis of exclusion - hemosiderrin macrophages are seen but none of the symptoms of Goodpasture Syndrome or Wegener Granulomatosis. And the cause of the depostis is not known.
• This diagnosis is fairly rare but it seems to be made more in children than adults.

Question 11

Describe the histopathology and treatment for Idiopathic Pulmonary Hemosiderosis.

Answer 11

• Histopathology is similar to Goodpasture but with no associated renal disease or anti-BM antibodies
• Treated with steroids and immunosuppression

Question 12

What type of cancer comprises 90-95% of all lung cancers?

Answer 12

Bronchiogenic carcinoma

Question 13

What genetic mutation is seen in almost all cases of lung cancers?

Answer 13

3p del

(deletion of the short arm (p) of the 3rd chromosome)

Question 14

What are the lung symptoms lung cancer can cause?

Answer 14

• If peripheral, the tumor can grow rather large before eliciting symptoms
• Total obstruction of airway leading to atelectasis and infection
• Partial obstruction of airway leading to infection
• Cough
• Hemoptysis
• Dyspnea
• Pleural effusion

Question 15

What non-lung related symptoms can lung cancers cause?

Answer 15

• Cachexia
• Pulmonary osteoarthropathy (clubbing from hypoxia)
• Hoarseness (irritation/paralysis of recurrent laryngeal)
• Chest Pain, Pericardial/Pleural Effusion (irritation of linings)
• Superior Vena Cava syndrome (compression of SVC)
• Horner syndrome (compression of cervical SNS plexus)
• Wasting of hand muscles and pain in arms (T1/T2 compression)
• Periperal Edema (BV compression)
• Dysphagia
• Chylothorax (thoracic duct obstruction)
• Paraneoplastic syndromes
• Metastasis related symptoms

Question 16

What is SVC Syndrome?

Answer 16

Edema of the face and arms caused by compression of the SVC

Question 17

What is horner syndrome?

Answer 17

A collection of symptoms caused by cervical SNS plexus damage

• Ipsilater enophthalmos (sunken eye)
• Ptosis
• Meiosis
• Anhidrosis

Question 18

What are paraneoplastic syndromes and why are they clinically important?

Answer 18

They’re symptoms that occur in patients with cancer that cannot be readily explained by mass effects. Usually caused by tumor secretion of cytokines/hormones or by immune reactions.

These are very important clinically because they’re often the earliest manifestation of a tumor, they can cause significant problems that are often lethal, they may mimic metastases, and they’re difficult to treat.

Question 19

List and describe the paraneoplastic syndromes we need to know and the type of lung cancer they’re most frequently associated with.

Answer 19

• Hypercalcemia - secretion of PTH - squamous cell carcinoma
• The rest are all most associated with small cell carcinoma
  
  • Cushing’s Syndrome - ACTH secretion
  • SIADH (hyponatremia) - ADH secrtion
  • Myasthenic Like Syndrome (eaton lambert) - auto immune reaction

Question 20

What areas of the body are most often affected by lung cancer metastasis?

Answer 20

1. Lymph nodes
2. Adrenals
3. Liver
4. Brain
5. Bone