Anti-Neutrophil Cytoplasmic Antibody (Anca)

Anti-Neutrophil Cytoplasmic Antibody (Anca)

Q. 1

Which of the following types of hepatitis is associated with an immune-mediated vasculitis characterized by p-ANCA antibodies?

 A

Hepatitis A

 B

Hepatitis B

 C

Hepatitis C

 D

Hepatitis D

Q. 1

Which of the following types of hepatitis is associated with an immune-mediated vasculitis characterized by p-ANCA antibodies?

 A

Hepatitis A

 B

Hepatitis B

 C

Hepatitis C

 D

Hepatitis D

Ans. B

Explanation:

Hepatitis B is associated with polyarteritis nodosa (PAN), a necrotizing vasculitis of small- and medium-sized muscular arteries involving all organ systems.

A significant percentage of patients with PAN have hepatitis B antigenemia.

They also have circulating immune complexes containing hepatitis B antigen.

Hepatitis B antigen, IgM, and complement can be demonstrated in blood vessel walls. P-ANCA (perinuclear-anti nuclear cytoplasmic antibody) is a marker for polyarteritis nodosa.

Hepatitis A is not associated with a vasculitis. Hepatitis C accounts for 50-70% of chronic hepatitis. Chronic hepatitis C can be associated with immune-complex mediated extrahepatic complications, but is less common than hepatitis B.

Hepatitis C does have a significant association with essential mixed cryoglobulinemia, which presents with glomerulonephritis, arthralgias, hepatosplenomegaly, and lymphadenopathy, in addition to a vasculitis. However, there is no association with p-ANCA.

Hepatitis D requires that the patient be co-infected with hepatitis B. As such, it does not independently cause disease, but it can produce a worsening of the liver disease.


Q. 2

C- ANCA positivity indicates, antibody formed against which of the following?

 A

Proteinase 3

 B

Myeloperoxidase

 C

Cytoplasmic antinuclear Antibody

 D

Anti centromere Antibody

Q. 2

C- ANCA positivity indicates, antibody formed against which of the following?

 A

Proteinase 3

 B

Myeloperoxidase

 C

Cytoplasmic antinuclear Antibody

 D

Anti centromere Antibody

Ans. A

Explanation:

C-ANCAs (Cytoplasmic anti-neutrophil cytoplasmic antibodies), are a type of autoantibody formed against proteinase 3.

P-ANCA (Perinuclearanti-neutrophil cytoplasmic antibodies) are antibodies directed against myeloperoxidase.

Ref: Harrison’s Principles of Internal Medicine, 16th Edition, Page 2002; Thurlbeck’s Pathology Of The Lung, William M. Thurlbeck, 3rd Edition, Page 372; Rheumatology Secrets, Sterling G. West, 2nd Edition, Page 226.

 


Q. 3

All are TRUE about Antineutrophil Cytoplasmic Antibodies, EXCEPT:

 A

Proteinase-3, in neutrophil azurophilic granules, is the major cANCA antigen

 B

The major target for pANCA is the enzyme myeloperoxidase

 C

A pANCA pattern of staining has been associated with nonvasculitic entities such as rheumatic and nonrheumatic autoimmune diseases

 D

There are three major categories of ANCA

Q. 3

All are TRUE about Antineutrophil Cytoplasmic Antibodies, EXCEPT:

 A

Proteinase-3, in neutrophil azurophilic granules, is the major cANCA antigen

 B

The major target for pANCA is the enzyme myeloperoxidase

 C

A pANCA pattern of staining has been associated with nonvasculitic entities such as rheumatic and nonrheumatic autoimmune diseases

 D

There are three major categories of ANCA

Ans. D

Explanation:

There are two major categories of ANCA. Other statements are true.

 
Ref: Harrisons Principles of Internal Medicine, 18th Edition, Page 2786

Quiz In Between


Q. 4

ANCA associated with Wegner’s granulomatosis‑

 A

cANCA

 B

pANCA

 C

Both

 D

None

Q. 4

ANCA associated with Wegner’s granulomatosis‑

 A

cANCA

 B

pANCA

 C

Both

 D

None

Ans. A

Explanation:

Ans. is ‘a’ i.e., cANCA

Antineutrophil cytoplasmic antibodies (ANCA)

o ANCA are heterogenous group of autoantibodies directed against antigens which are found within the primary granules of neutrophil and in the lysosomes of monocytes and in EC’s.

o The description of these autoantibodies is based on the immunofluorescence pattern of staining of ethanol fixed neutrophils. With immunofluorescence two principal patterns are recognized:

1.  Cytoplasmic (c-ANCA):

o This shows cytoplasmic localization of the staining and the target antigen for this type of autoantibody is — proteinase-3 (PR3), a neutrophil granule constituent.

2.  Perinuclear staining (p-ANCA)

o This shows perinuclear staining and the target antigen here is myeloperoxide (MPO).

Remember that either of these antibodies may occur in a patient with ANCA associated small vessel vasculitis but

(i) cANCA  Typically found in

  • Wegener’s granulomatosis

(ii) pANCA  Typically found in

  • Microscopic polyangiitis

o Churg-Strauss syndrome

o Idiopathic crescentic glomerulonephritis

o Goodpasteur’s syndrome.

o pANCA’s are also associated with certain non-vasculitic entities such as certain rheumatic and nonrheumatic autoimmune diseases, Inflammatory bowel diseases, certain drugs, • infections such as endocarditis and • bacterial airway infection in patients with cystic fibrosis.


Q. 5

pANCA is sensitive and specified for-

 A

Post streptococcal glomerulonephritis

 B

Idiopathic cresentic glomerulonephritis

 C

Diffuse glomerulosclerosis

 D

Diffuse glomerulosclerosis

Q. 5

pANCA is sensitive and specified for-

 A

Post streptococcal glomerulonephritis

 B

Idiopathic cresentic glomerulonephritis

 C

Diffuse glomerulosclerosis

 D

Diffuse glomerulosclerosis

Ans. B

Explanation:

Ans. is ‘b’ i.e., Idiopathic crecsentric glomerulonephritis


Q. 6

ANCA positive vasculitis

 A

Henoch schonlein purpura

 B

Behcet’s syndrome

 C

Wegener’s granulomatosis

 D

All

Q. 6

ANCA positive vasculitis

 A

Henoch schonlein purpura

 B

Behcet’s syndrome

 C

Wegener’s granulomatosis

 D

All

Ans. C

Explanation:

Ans. is ‘c’ i.e., Wegener’s granulomatosis

Quiz In Between


Q. 7

ANCA positive vasculitis are –

 A

Churg-strauss syndrome

 B

Polyarteritis nodosa

 C

Wegener granulomatosis

 D

a and c

Q. 7

ANCA positive vasculitis are –

 A

Churg-strauss syndrome

 B

Polyarteritis nodosa

 C

Wegener granulomatosis

 D

a and c

Ans. D

Explanation:

Ans. is ‘a’ i.e., Churg-strauss syndrome; ‘c’ i.e., Wegener granulomatosis


Q. 8

ANCA positive is –

 A

Good pasture syndrome

 B

Wegner’s granulomatosis

 C

Sjogren syndrome

 D

a and b

Q. 8

ANCA positive is –

 A

Good pasture syndrome

 B

Wegner’s granulomatosis

 C

Sjogren syndrome

 D

a and b

Ans. D

Explanation:

Ans. is ‘a’ i.e., Good pasture syndrome; ‘b’ i.e., Wegner’s granulomatosis

Amongst the given options two are associated with ANCA: Wegner’s granulomatosis is positive for cANCA and goodpasture syndrome is positive for pANCA.


Q. 9

A patient presents with respiratory symptoms i.e. cough, hemoptysis and glomerulonephritis. His C-­ANCA levles in serum were found to be raised. The most likely diagnosis is –

 A

Goodpasteur’s syndrome

 B

Classic polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Kawasaki syndrome

Q. 9

A patient presents with respiratory symptoms i.e. cough, hemoptysis and glomerulonephritis. His C-­ANCA levles in serum were found to be raised. The most likely diagnosis is –

 A

Goodpasteur’s syndrome

 B

Classic polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Kawasaki syndrome

Ans. C

Explanation:

Ans. is ‘c’ i.e., Wegener’s granulomatosis

o Renal and pulmonary symptoms with positive c-ANCA suggest the diagnosis of wegener’s glomerulonephritis (see above explanation).

o Clinical syndrome of wegener granulomatosis may be very similar to classical PAN or microscopic polyangitis. Following facts will help you to differentiate wegner granulomatosis from other Two ‑

Wegeners granulomatosis Vs classical PAN

o Wegener’s granulomatosis involves small vessels (arteriole, venule, capillary), while PAN involves medium size vessels.

o Wegener’s granulomatosis is associated with c-ANCA, while classical PAN is not associated with ANCA. o In wegener’s granulomatosis there is lung involvement, but not in classical PAN.

o In wegener’s granulomatosis there is glomerulonephritis (hematuria) without hypertension, while in classical PAN there is hypertension without glomerulonephritis.

o In wegener’s granulomatosis, there is granuloma formation, while it is absent in classical PAN. o Classical PAN shows microaneurysms which are absent in wegener’s granulomatosis.

Wegner’s granulomatosis Vs microscopic polyangitis

o Wegener’s granulomatosis is associated with c-ANCA, while MPA is associated with p-ANCA. o Granuloma formation occurs in wegener’s granulomatosis, not in MPA.

o Lung cavities and nodules are seen in wegener’s granulomatosis, but not in MPA.

Quiz In Between


Q. 10

Which of the following condition(s) are associated with raised ANCA except –

 A

Wegener’s granulomatosis

 B

Polyarteritis Nodosa

 C

Microscopic Polyangitis

 D

Churg-Strauss syndrome

Q. 10

Which of the following condition(s) are associated with raised ANCA except –

 A

Wegener’s granulomatosis

 B

Polyarteritis Nodosa

 C

Microscopic Polyangitis

 D

Churg-Strauss syndrome

Ans. B

Explanation:

Ans. is ‘b’ i.e., Polyarteritis Nodosa


Q. 11

All are true about ANCA associated crescentic glomerulonephritis, except –

 A

Seen in Wegner’s granulomatosis

 B

Seen in microscopic polyangitis

 C

Seen in Henoch Schonlein purpura

 D

Is pauci-immue in nature

Q. 11

All are true about ANCA associated crescentic glomerulonephritis, except –

 A

Seen in Wegner’s granulomatosis

 B

Seen in microscopic polyangitis

 C

Seen in Henoch Schonlein purpura

 D

Is pauci-immue in nature

Ans. C

Explanation:

Ans. is ‘c’ i.e., Seen in Henoch Schonlein purpura


Q. 12

A 20 year old male presents with mucus and repeated gastrointestinal bleeding. Patient is positive for ANCA. The most likely diagnosis is:

 A

Ulcerative colitis

 B

Crohn’s disease

 C

Radiation colitis

 D

Ischemic bowel disease

Q. 12

A 20 year old male presents with mucus and repeated gastrointestinal bleeding. Patient is positive for ANCA. The most likely diagnosis is:

 A

Ulcerative colitis

 B

Crohn’s disease

 C

Radiation colitis

 D

Ischemic bowel disease

Ans. A

Explanation:

Answer is A (Ulcerative Colitis)

Presence of mucus and blood in stool along with positive ANCA antibodies suggests a diagnosis of ulcerative colitis

Different Clinical, Endoscopic and Radiographic Features

 Clinical

Ulcerative Colitis

Crohn’s Disease

 

Gross blood in stool

Yes

Occasionally

Occasionally

Mucus

Yes

Systemic symptoms

Occasionally

Frequently

Pain

Occasionally

Frequently.

Abdominal mass

Rarely

Yes

Significant perineal disease

No

Frequently

Fistulas

No

Yes

Small-intestinal obstruction

No

Frequently

Colonic obstruction

Rarely

Frequently

Response to antibiotics

No

Yes

Recurrence after surgery

No

Yes

ANCA-positive

Frequently

Rarely

ASCA —positive

Rarely

Frequently

Endoscopic

Rectal sparing

Rarely

Frequently

Continuous disease

Yes

Occasionally

Cobblestoning

No

Yes

Granuloma on biopsy

No

Occasionally

Radiographic

Small bowel significantly abnormal

No

Yes

Abnormal terminal ileum

Occasionally

Yes

Segmental colitis

No

Yes

Asymmetric colitis

No

Yes

Stricture

Occasionally

Frequently

Epidemiology of 1BD

Age of onset

15-30 and 60-80

15-30 & 60-80

Male : Female ratio

1:1

“4.5 : 1

Smoking

May prevent disease

May cause disease

Oral contraceptives

No increased risk

Odds ratio 1.4

Appendectomy

Protective

Not protective

Monozygotic twins

6% concordance

58% concordance

Dizygotic twins

0% concordance

4% concordance

Quiz In Between


Q. 13

A patient presents with hemoptysis and hematuria few weeks after a respiratory tract infection.

ANCA antibodies are present.

Likely diagnosis is:

 A

Goodpasture’s syndrome

 B

IgA Nephropathy

 C

Nephrotic syndrome

 D

PSGN

Q. 13

A patient presents with hemoptysis and hematuria few weeks after a respiratory tract infection.

ANCA antibodies are present.

Likely diagnosis is:

 A

Goodpasture’s syndrome

 B

IgA Nephropathy

 C

Nephrotic syndrome

 D

PSGN

Ans. A

Explanation:

Answer is A (Goodpasture’s syndrome)

  • Association of hematuria and hemoptysis suggest a diagnosis of Goodpasture’s syndrome.

Goodpasture’s syndrome and ANCA

  1. ANCA is typically negative in Goodpastures syndrome and it is classified as an ANCA negative vasculitis

Nevertheless

  • ANCA seropositivity may occur in upto 30 percent of patients with Goodpasture’s.syndrome.
  • These patients with ANCA have subtle signs of a systemic vasculitis and represent an overlap between goodpastures and either Wegener’s granulomatosis (WG) or Microscopic polyangitis(MPA)-Current Diagnosis and Treatment in Pulmonary Medicine

 


Q. 14

ANCA is found in all of the following Except:

 A

A. Wegner’s granulomatosis

 B

Churg-Strauss disease

 C

Microscopic polyangitis

 D

Takayasu arteritis

Q. 14

ANCA is found in all of the following Except:

 A

A. Wegner’s granulomatosis

 B

Churg-Strauss disease

 C

Microscopic polyangitis

 D

Takayasu arteritis

Ans. D

Explanation:

Answer is D (Takayasu Arteritis):

Takayasu Arteritis is not associated with ANCA

Note:

If p-ANCA is not against Myeloperoxidase (or CANCA is not against Proteinase-3), look for causes other than Vasculitis for the positive ANCA.


Q. 15

ANCA positive vasculitis include all of the following Except:

 A

Wegner’s granulomatosis

 B

Churg strauss syndrome

 C

Microscopic PAN

 D

Good pasture’s syndrome

Q. 15

ANCA positive vasculitis include all of the following Except:

 A

Wegner’s granulomatosis

 B

Churg strauss syndrome

 C

Microscopic PAN

 D

Good pasture’s syndrome

Ans. D

Explanation:

Answer is D (Good pasture’s syndrome):

Goodpasture’s syndrome is not classified as an ANCA positive vasculitis.

Quiz In Between


Q. 16

ANCA is NOT associated with which of the following disease:

 A

Wegener’s Granulomatosis

 B

Henoch Schonlein Purpura

 C

Microscopic Polyangitis

 D

Churg Strauss syndrome

Q. 16

ANCA is NOT associated with which of the following disease:

 A

Wegener’s Granulomatosis

 B

Henoch Schonlein Purpura

 C

Microscopic Polyangitis

 D

Churg Strauss syndrome

Ans. B

Explanation:

Answer is B (Henoch Schonlein Purpura):

Henoch Schonlein Purpura is an ANCA Negative Vasculitis


Q. 17

ANCA is NOT associated with which of the following diseases :

 A

Wegener’s granulomatosis

 B

Henoch schonlein purpura

 C

Microscopic PAN

 D

Churg Strauss syndrome

Q. 17

ANCA is NOT associated with which of the following diseases :

 A

Wegener’s granulomatosis

 B

Henoch schonlein purpura

 C

Microscopic PAN

 D

Churg Strauss syndrome

Ans. B

Explanation:

Answer is B (H.S. Purpura) :

H.S. purpura is not associated with any antinuclear cytoplasmic antibody (ANCA). It is an example of ANCA negative vasculitis.

  • ANCA (Antineutrophilic cytoplasmic Antibodies) arc Antibodies directed against certain proteins in cytoplasmic granules of Neutrophil & monocytes.
  • These are two major categories of ANCA based on different targets for the antibodies.

ANCA (Antineutrophic cytoplasmic antibodies):

ANCA is of 2 types

C-ANCA

(Cytoplasmic proteinase 3q is the target antigen)

Wegeners Granulomatosisq (90-95%)

P-ANCA

(perinuclear myeloperoxidaseQ is the major target antigen)

  • Microscopic PAN (microscopic polyangitis)
  • Churg-Strauss syndrome
  • Crescenteric glomerulonephritise
  • Good pasteur’s syndrome

Q. 18

CANCA Positivity is specific for:

 A

Polyarteritis Nodosa

 B

Wegener’s granulomatosis

 C

Henoch shontein purpura

 D

Churg strouss syndrome

Q. 18

CANCA Positivity is specific for:

 A

Polyarteritis Nodosa

 B

Wegener’s granulomatosis

 C

Henoch shontein purpura

 D

Churg strouss syndrome

Ans. B

Explanation:

Answer is B (Wegener’s granulomatosis):

CANCA refers to Cytoplasmic Antineutrophilic cytoplasmic antibodies and its presence is highly specific for Wegener’s granulomatosis

Quiz In Between


Q. 19

c-ANCA is characteristic for :

 A

Polyarteritis Nodosa

 B

R.P.G.N.

 C

Henoch’s Schonlein Purpura

 D

Wegeners granulomatosis

Q. 19

c-ANCA is characteristic for :

 A

Polyarteritis Nodosa

 B

R.P.G.N.

 C

Henoch’s Schonlein Purpura

 D

Wegeners granulomatosis

Ans. D

Explanation:

Answer is D (Wegener’s granulomatosis) :

Antineutrophilic cytoplasmic antibody directed against cytoplasmic proteinase-3 (cANCA) is found in 90-95% if patients with Wegener’s granulomatosis.


Q. 20

C-ANCA is associated with:

 A

Wegener’s Granulomatosis

 B

Microscopic Polyangitis

 C

Churg- Strauss Syndrome

 D

Polyarteritis Nodosa (PAN)

Q. 20

C-ANCA is associated with:

 A

Wegener’s Granulomatosis

 B

Microscopic Polyangitis

 C

Churg- Strauss Syndrome

 D

Polyarteritis Nodosa (PAN)

Ans. A

Explanation:

Answer is A (Wegener’s Granulomatosis):

cANCA refers to Cytoplasmic Antineutrophilic Cytoplasmic Antibodies and its presence is highly specific for Wegener’s Granulomatosis. Antineutrophilic Cytoplasmic Antibody directed against cytoplasmic. proteinase-3 (cANCA) is found in 90-95% if patients with Wegener’s Granulomatosis.


Q. 21

c-ANCA positivity indicates, antibody formed against:

 A

Proteinase 3

 B

Myeloperoxidase

 C

Cytoplasmic antinuclear antibody

 D

Anti centromere antibody

Q. 21

c-ANCA positivity indicates, antibody formed against:

 A

Proteinase 3

 B

Myeloperoxidase

 C

Cytoplasmic antinuclear antibody

 D

Anti centromere antibody

Ans. A

Explanation:

Answer is A (Proteinase 3):

Proteinase – 3 (a 29 – kDA serine proteinase) present in neutrophil azurophilic granules is the major c-ANCA antigen.

Major c-ANCA antigen        Proteinase 3 (PR-3)

Major p – ANCA antigen    Myeloperoxidase (MPO)

Quiz In Between


Q. 22

p-ANCA is characteristic for:

 A

PAN

 B

Microscopic polyangitis

 C

Wegener’s granulomatosis

 D

Henoch-Schonlein purpura

Q. 22

p-ANCA is characteristic for:

 A

PAN

 B

Microscopic polyangitis

 C

Wegener’s granulomatosis

 D

Henoch-Schonlein purpura

Ans. B

Explanation:

Answer is B (Microscopic polyangitis) :

PAN can broadly be classified into

Classic PAN
(now referred to an PAN)

  • Classified as Medium Vessel Vasculitis
  • Necrotizing inflammation of small & medium sized arteries.
  • No involvement of capillaries & venules
  • Pulmonary vasculature is not involved.
  • ANCA mostly negative (Rarely p-ANCA may be seen)

Microscopic PAN
(now called microscopic polyangitis)

  • Classified as small vessel vasculitis
  • Necrotizing inflammation of small & medium sized arteries.
  • Capillaries & venules are involved
  • Pulmonary vasculature is involved.
  • ANCA positive (p-ANCA)



Q. 23

All of the following are true regarding Wegener’s granulomatosis except: 

September 2008

 A

Affects large size blood vessels

 B

May affect lungs and kidneys

 C

Necrotizing granuloma may be seen on microscopy

 D

PR3-ANCAs are present in upto 95% of cases

Q. 23

All of the following are true regarding Wegener’s granulomatosis except: 

September 2008

 A

Affects large size blood vessels

 B

May affect lungs and kidneys

 C

Necrotizing granuloma may be seen on microscopy

 D

PR3-ANCAs are present in upto 95% of cases

Ans. A

Explanation:

Ans. A: Affects large size blood vessels

Wegener’s granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs.

Due to its end-organ damage, it is life-threatening and requires long-term immunosuppression.

Wegener’s granulomatosis is part of a larger group of vasculitic syndromes, all of which feature an autoimmune attack by an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) against small and medium-size blood vessels.

Apart from Wegener’s, this category includes Churg-Strauss syndrome and microscopic polyangiitis.

Cytoplasmic staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils are associated with Wegener’s (upto 95% of cases).

If the patient has renal failure or cutaneous vasculitis, these are the most logical organs to obtain a biopsy from.

On histopathological examination, a biopsy will show leukocytoclastic vasculitis with necrotic changes and granulomatous inflammation on microscopy. These granulomas are the main reason for the appellation of “Wegener’s granulomatosis”, although it is not an essential feature.


Q. 24

ANCA associated with Wegner’s granulomatosis‑

 A

cANCA

 B

pANCA

 C

Both

 D

None

Q. 24

ANCA associated with Wegner’s granulomatosis‑

 A

cANCA

 B

pANCA

 C

Both

 D

None

Ans. A

Explanation:

Ans. is ‘a’ i.e., cANCA

Quiz In Between


Q. 25

pANCA is sensitive and specified for‑

 A

Post streptococcal glomerulonephritis

 B

Idiopathic cresentic glomerulonephritis

 C

Diffuse glomerulosclerosis

 D

Diffuse glomerulosclerosis

Q. 25

pANCA is sensitive and specified for‑

 A

Post streptococcal glomerulonephritis

 B

Idiopathic cresentic glomerulonephritis

 C

Diffuse glomerulosclerosis

 D

Diffuse glomerulosclerosis

Ans. B

Explanation:

Ans. is ‘b’ i.e., Idiopathic cresentic glomerulonephritis


Q. 26

CANCA is associated with ‑

 A

Wegener’s granulomatosis

 B

Microscopic polyangitis

 C

Chrug strauss syndrome

 D

Good pasture syndrome

Q. 26

CANCA is associated with ‑

 A

Wegener’s granulomatosis

 B

Microscopic polyangitis

 C

Chrug strauss syndrome

 D

Good pasture syndrome

Ans. A

Explanation:

Ans. is ‘a’ i.e., Wegener’s granulomatosis

Quiz In Between



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