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Tutorials > CPC respiratory/vasculitis > Flashcards

CPC respiratory/vasculitis Flashcards

1
Q

What is a pericardial rub

A

High-pitched scratching sound best heard on auscultation over the left sternal border during expiration while the patient is sitting up and leaning forward The rub is heard in atrial and ventricular systole as well as early diastole. It indicates friction between the visceral and parietal pericardial tissue. Seen most commonly in pericarditis.

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2
Q
  • -Respiratory, renal and joint disease with a high ESR.

- -What differential diagnosis would one consider?

A

Best fit for multi-system disease (Renal, lung, and Joints) is
Immune-mediated

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3
Q

right-sided chest pain which was worse on taking a deep breath indicates…

A

pleuritis or pericarditis

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4
Q

crackles on lung auscultation are concerning for…

A

A pathologic breath sound on auscultation characterized by discontinuous, intermittent rattling. Etiologies include pneumonia, atelectasis, pulmonary fibrosis, bronchiectasis, interstitial lung disease, and pulmonary edema.

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5
Q

what is the ESR?

A

A test that measures the distance erythrocytes fall after one hour in a vertical tube of anticoagulated blood. Can be elevated in many conditions such as infection, inflammation, and malignancy.

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6
Q

what are the causes of elevated ESR?

A
  • -Elevated fibrinogen level (infection, inflammation, malignancy)
  • -Pregnancy (↑ fibrinogen)
  • -Old age
  • -Anemia, macrocytosis
  • -Multiple myeloma, Waldenstrom macroglobulinemia
  • -Autoimmune diseases (systemic lupus erythematosus, rheumatoid arthritis, giant cell arteritis, polymyalgia rheumatica, de Quervain thyroiditis)
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7
Q

how fibrinogen increases ESR?

A

Fibrinogen coats the surface of RBCs and reduces their charges. As a result, they aggregate more rapidly.

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8
Q

what are the causes of normocytic anemia?

A

A type of anemia that is characterized by red blood cells of normal size (with a mean corpuscular volume of 80–100 fL). Etiologies include hemolytic anemias, acute bleeding, and early anemia of chronic disease.

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9
Q

sputum cytology used for diagnosis of…

A

lung cancer, asthma, TB, pneumonia

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10
Q

What investigations should be performed to assess immune-based etiology?

A

1) Autoantibody screen
- -E.g. ANA, RF
- -ANCAs: Anti-neutrophil cytoplasmic antibodies
- -Anti GBM
2) Complement levels

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11
Q

what is the ANA?

A

An autoantibody against the cell nucleus that can be present in several autoimmune conditions, including systemic lupus erythematosus and systemic sclerosis.

  • -ANAs are comprised of numerous antibodies against specific nuclear antigens.
  • -In addition, ANAs include autoantibodies against DNA and histones.
  • -An increase is typically observed in connective tissue diseases, but may also be found in, e.g., rheumatoid arthritis (RA), autoimmune hepatitis, and vasculitides.
  • -In general, elevated ANA levels are considered nonspecific; exact diagnosis requires determination of individual antibodies.
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12
Q

examples of ANA specific for SLE?

A
  • -Anti-dsDNA (in ∼ 70% of cases)
  • -Anti-Sm (Smith)
  • -Anti-histone (in drug-induced SLE)
  • -Anti-Ro/SSA
  • -Anti-La/SSB
  • -Anti-U1 RNP (ribonucleoprotein)
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13
Q

examples of ANA specific for SS?

A

anti-Scl-70 (anti-topoisomerase antibody I: seen in 30–70% of cases

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14
Q

examples of ANA specific for Sjögren syndrome?

A
  • -Anti-Ro/SSA
  • -Anti-La/SSB
  • -60–80% of patients positive for one or both antibodies
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15
Q

examples of ANA specific for Polymyositis and dermatomyositis?

A
  • -Anti-Jo1

- -Anti-Mi2

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16
Q

examples of ANA specific for Mixed connective tissue disease?

A

anti-U1-RNP

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17
Q

what is the ANCA?

A

An antibody against specific cytoplasmic antigens. An increase in ANCAs is a common finding in autoimmune vasculitides (e.g., p-ANCA in eosinophilic granulomatosis with polyangiitis, c-ANCA in granulomatosis with polyangiitis)

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18
Q

c-ANCA (Proteinase-3 antibody) is seen in…

A
  • -Granulomatosis with polyangiitis (Wegener granulomatosis)

- -90% of patients are c-ANCA positive.

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19
Q

p-ANCA (Myeloperoxidase antibody) is seen in…

A
  • -Microscopic polyangiitis: ∼ 70% of patients are ANCA positive (mostly p-ANCA).
  • -Churg-Strauss syndrome: ∼ 50% of patients are ANCA positive (both c-ANCA and p-ANCA).
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20
Q

perinuclear vs cytoplasmic ANCA?

A

p-ANCA vs c-ANCA

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21
Q

what are the 3 primary diseases consistently associated with ANCA positive vasculitis?

A
  • -Granulomatosis with polyangiitis (Wegener’s granulomatosis),
  • -microscopic polyangiitis
  • -glomerulonephritis.
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22
Q

The clinical evidence of multi-system involvement with an elevated c-ANCA is suggestive of a diagnosis of a…

A

vasculitic disease.

23
Q

does positive ANCA is significant in the absence of clinical signs and symptoms?

A

ANCA results should not be interpreted in the absence of signs, symptoms, or other objective evidence of disease

24
Q

what is the treatment for ANCA positive Granulomatosis polyangiitis (AKA Wegener’s granulomatosis)

A

Steroids and cyclophosphamide

25
Q

Why was necrosis seen in Chest X-Ray of a patient with Wegener’s granulomatosis?

A

Due to tissue necrosis due to ischemia secondary to vasculitis

26
Q

In Wegner’s disease (Granulomatosis with vasculitis)nodules in the lungs are ‘cavitating’ due to necrosis. True/False

A

True

Chest x-ray/CT: multiple bilateral cavitating nodular lesions

27
Q

what is vasculitis?

A

A vasculitis is primary inflammation of the vessel wall with a variety of inflammatory cells (neutrophils, lymphocytes, eosinophils, histiocytes, giant cells) +/- fibrinoid necrosis.

28
Q

what is the fibrinoid necrosis?

A

A type of necrosis characterized by vessel wall damage caused by immune complexes that combine with fibrin (type III hypersensitivity reaction). See on histological examination of vessels in patients with polyarteritis nodosa, preeclampsia, and hypertensive emergency.

29
Q

in vasculitides, the vessels in many different sites are involved. Most commonly in…

A
  • -skin, lungs, kidneys and CNS, GIT, etc.

- -hence the multi-system clinical manifestations of the disease

30
Q

what are the vasculitides involving large vessels?

A

giant cell arteritis, polymyalgia rheumatica, Takayasu’s arteritis

31
Q

what are the vasculitides involving medium vessels?

A

cutaneous vasculitis, polyarteritis nodosa, Kawasaki disease

32
Q

what are the vasculitides involving small vessels?

A

Churg-Strauss syndrome, cutaneous vasculitis, Henoch-Schönlein purpura, microscopic polyangiitis, (previously was included in PAN), Granulomatosis with polyangiitis (Wegener’s)

33
Q

ANCA mostly positive in small and large vessel diseases, T/F

A

ANCA mostly positive in small and medium-sized vessel diseases (not usually in IgA/HSP)

34
Q

how vasculitides are diagnosed?

A

1) Laboratory tests
- -ESR, CRP, anemia, raised WCC, eosinophilia
- -Elevated ANCA (in some)
- -Haematuria
2) Biopsy of involved organs
- -Diagnosis generally confirmed with tissue biopsy from a site of active disease e.g. Skin, lung, kidney, sinuses (especially kidney)
- -Demonstrates size of vessels involved and type of inflammation

35
Q

how a diagnosis of vasculitides is confirmed?

A
  • -Diagnosis generally confirmed with tissue biopsy from a site of active disease e.g. Skin, lung, kidney, sinuses (especially kidney)
  • -Demonstrates size of vessels involved and type of inflammation
36
Q

what is Wegener’s granulomatosis?

A

Wegener’s granulomatosis (Granulomatosis with angiitis) is part of a larger group of vasculitic syndromes, all of which feature an autoimmune attack by the abnormal circulating antibody (ANCA’s) against small and medium-size blood vessels.

37
Q

which vessels are primarily involved in Wegener’s?

A

In Wegener’s, the vessels of the upper and lower airways of the lungs and the kidneys are primarily involved

38
Q

what is the pathology of Wegener’s granulomatosis?

A
  • -Necrotic, partially granulomatous vasculitis of small and medium-sized vessels
  • -Necrotizing granulomas (intravascular and extravascular)
  • -Glomerulonephritis
39
Q

what are the other vasculitides involving the lung?

A
  • -Polyarteritis nodosa (PAN)
  • -Microscopic polyarteritis (previously part of PAN)
  • -Churg-Strauss Syndrome
  • -Rheumatoid Arthritis (pleural effusion)
40
Q

what are the kidney manifestations of Wegener’s?

A

Pauci-immune glomerulonephritis (Pauci‑immune indicates that there is little evidence of immune complex/antibody deposits.) → rapidly progressive (crescentic) glomerulonephritis (RPGN), with possible pulmonary-renal syndrome

41
Q

what are the ENT manifestations of Wegener’s?

A
  • -ENT involvement(∼ 90% of cases): often the first clinical manifestation
    1) Chronic rhinitis/sinusitis: nasopharyngeal ulcerations → nasal septum perforation → saddle nose deformity (depression of the nasal dorsum)
    2) In some cases, thick, purulent discharge, sometimes containing blood
    3) Oral ulcers
    4) Chronic otitis
    5) Gingival hyperplasia (strawberry gingivitis)
    6) underlying bone destruction with a loosening of teeth
    7) conductive hearing loss due to auditory tube dysfunction, sensorineural hearing loss (an unclear mechanism)
    8) eyes: pseudotumors, scleritis, conjunctivitis, uveitis, episcleritis
42
Q

which monoclonal antibody is used in the treatment of Wegener’s?

A
  • -The introduction of cyclophosphamide (CYC) greatly improved the prognosis
  • -Monoclonal antibody that targets B cells (Rituximab) has been approved for Wegner’s disease
  • -Five-year survival is now ~ 87%.
43
Q

what are the Immune-mediated disease of the lung?

A
  • -Wegener’s (Granulomatosis polyangiitis)
  • -Churg-Strauss Syndrome
  • -Goodpasture’s syndrome
  • -PAN rare
44
Q

what is the Goodpasture’ssyndrome?

A

A rare autoimmune disease characterized by circulating antibodies against the alpha-3 chain of type IV collagen present in the glomerular basement membrane and pulmonary capillary basement membranes. Can cause a spectrum of disease, ranging from minimally symptomatic disease to pulmonary-renal syndrome with hemoptysis (from alveolar hemorrhage), cough, dyspnea, and rapidly progressive glomerulonephritis.

45
Q

what is the Churg-Strauss syndrome?

A

A necrotizing, granulomatous small vessel vasculitis that commonly manifests with pulmonary (e.g., asthma, allergic rhinitis) and skin (e.g., purpura or tender nodules) involvement. Can affect the intestines (e.g., bleeding, ischemia, perforation), heart (e.g., pericarditis), and/or kidneys (e.g., focal segmental necrotizing glomerulonephritis). Associated with peripheral eosinophilia, increased IgE levels, p-ANCA positivity.

46
Q

what is the cor pulmonale?

A

Altered structure (hypertrophy, dilation) or impaired functioning of the right ventricle caused by a primary disorder of the respiratory system (e.g., COPD, cystic fibrosis, interstitial lung disease, pulmonary embolism). The primary respiratory disorder causes acute/chronic pulmonary hypertension, which in turn causes acute/chronic right heart failure.

47
Q

what are the differentials of chronic cor pulmonale?

A
  • -Idiopathic pulmonary fibrosis
  • -Hypersensitivity pneumonitis
  • -Congenital valvular heart disease
  • -Chronic episodes of pulmonary emboli
48
Q

what is the silicosis?

A

Silicosis is a common occupational lung disease that is caused by the inhalation of crystalline silica dust. Silica is the most abundant mineral on earth. Workers that are involved for example in constructions, mining, or glass production are among the individuals with the highest risk of developing the condition. Acute silicosis causes severe symptoms (e.g., exertional dyspnea, cough with sputum) and has a very poor prognosis. Chronic silicosis has a very variable prognosis and affected individuals may remain asymptomatic for several decades. However, radiographic signs are usually seen early on. Typical radiographic findings are calcifications of perihilar lymph nodes, diffuse ground glass opacities, large numbers of rounded, solitary nodules or bigger, confluent opacities.

49
Q

what investigations should be performed in suspected ILD and cor pulmonale?

A
  • -FBC
  • -Sputum – C&S
  • -CXR; probably progress to HRCT thorax
  • -PFT’s
  • -ABG
  • -ECG +/- ECHO
50
Q

65-year-old retired quarry working has a history of dyspnoea x 10 years. What is the most possible diagnosis?

A

silicosis

51
Q

PFT in silicosis is of obstructive or restrictive pattern?

A

restrictive

52
Q

list common types of pneumoconiosis?

A
  • -Silicosis – from silica dust
  • -Asbestosis – from asbestos dust
  • -Coal workers pneumoconiosis (anthracosis) – from coal dust
  • -Byssinosis – from cotton dust
  • -Bagassosis – from sugarcane dust
  • -Famer’s Lung – from hay dust or mould spores other agricultural products
  • -Berylliosis – from beryllium
53
Q

what is the hypersensitivity pneumonitis (extrinsic allergic alveolitis)?

A

An acute, subacute, or chronic pulmonary disease characterized by an immune-mediated inflammatory response in the alveoli and small airways as a result of exposure to a variety of inhaled antigens.

54
Q

what are the common causes of HSP?

A

1) organic dust (<5 mcm)
- -molds, avian proteins
2) some chemicals
- -diisocyanates
- -organic acid anhydrides

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