Granulomatosis With Polyangiitis (Wegener’s)

GRANULOMATOSIS WITH POLYANGIITIS (WEGENER’S)

Q. 1

In Wegeners glomerulonephritis characteristic feature seen in 

 A

Granuloma in the vessel wall

 B

Focal necrotizing glomerulonephritis

 C

Nodular glomerulosclerosis

 D

Interstitial granuloma

Q. 1

In Wegeners glomerulonephritis characteristic feature seen in 

 A

Granuloma in the vessel wall

 B

Focal necrotizing glomerulonephritis

 C

Nodular glomerulosclerosis

 D

Interstitial granuloma

Ans. B

Explanation:

Focal necrotizing glomerulonephritis [Ref. Harrison 17th/e p. 2121; Bobbin’s 8th/e p. 5161

  • Wegener’s granulomatosis is a distinct clinicopathologic entity characterized by granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis.
  • The histopathological hallmark of Wegener’s granulomatosis is necrotizing vasculitis of small arteries and veins together with granuloma formation which may be either intravascular or extravascular. An important point to note,
  • Though it is called granulomatous vasculitis:?

– Granulomas in Wegener’s’ granulomatosis are seen only in lungs or respiratory tract. Granuloma formation is rarely seen in other organs. Other organs, only demonstrate necrotizing vasculitis.

  • So Wegener’s granulomatosis can also be defined as Granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis.
  • Bobbin’s defines Wegener’s granulomatosis as “necrotizing vasculitis” (not granulomatous vasculitis). According to Robbin’s
  • Wegener’s granulomatosis is a necrotizing vasculitis characterized by a triad of 😕

– Acute necrotizing granuloma of upper respiratory tract (ear, nose, sinuses, throat) or the lower respiratory tract.

– Necrotizing or granulomatous vasculitis affecting small to medium sized vessels (e.g. capillaries, venules, arteriole and arteria) most prominent in the lungs and upper airways but affecting other sites as well. – Renal disease in the .form of focal necrotizing often crescenteric glomerulonephritis.

Glomerulonephritis in Wegener’s granulomatosis

  • Wegener’s glomerulonephritis is characterized by “focal and segmental necrotizing glomerulonephritis”. That may evolve into a rapidly progressive crescenteric glomerulonephritis.

Granuloma. formation is rarely seen in Wegener’s glomerulonephritis.


Q. 2

What proportion of Wegener’s will have antibodies to Proteinase-3?

 A

10%

 B

50%

 C

90%

 D

100%

Q. 2

What proportion of Wegener’s will have antibodies to Proteinase-3?

 A

10%

 B

50%

 C

90%

 D

100%

Ans. C

Explanation:

There are two types of ANCA based on different targets for the antibodies. 

Cytoplasmic ANCA (cANCA) is  the diffuse, granular cytoplasmic staining pattern observed by immunofluorescence microscopy when serum antibodies bind to indicator neutrophils. Proteinase-3 is the major c-ANCA antigen.
ALSO NOTE:
 
More than 90% of patients with typical active granulomatosis with polyangiitis (Wegener’s) have detectable antibodies to proteinase-3 
Ref: Harrison, E-18, P-2786.

Q. 3

A 45-year-old man presents to his physician with hematuria. Renal biopsy demonstrates a focal necrotizing glomerulitis with crescent formation. The patient has a history of intermittent hemoptysis and intermittent chest pain of moderate intensity. A previous chest x-ray had demonstrated multiple opacities, some of which were cavitated. The patient also has chronic cold-like nasal symptoms. Which of the following is the most likely diagnosis?

 A

Aspergillosis

 B

Polyarteritis nodosa

 C

Renal carcinoma metastatic to the lung

 D

Wegener’s granulomatosis

Q. 3

A 45-year-old man presents to his physician with hematuria. Renal biopsy demonstrates a focal necrotizing glomerulitis with crescent formation. The patient has a history of intermittent hemoptysis and intermittent chest pain of moderate intensity. A previous chest x-ray had demonstrated multiple opacities, some of which were cavitated. The patient also has chronic cold-like nasal symptoms. Which of the following is the most likely diagnosis?

 A

Aspergillosis

 B

Polyarteritis nodosa

 C

Renal carcinoma metastatic to the lung

 D

Wegener’s granulomatosis

Ans. D

Explanation:

While in real life, other diseases (or combinations of diseases) may occasionally cause concurrent pulmonary, sinus, and renal involvement, if you see this pattern on a test question, you should immediately think of Wegener’s granulomatosis.

This is a rare focal necrotizing vasculitis of still unclear etiology, which also features prominent granulomas, some of which are centered on the vascular lesions.
The vasculitis and granulomatous can involve the entire respiratory tract, and an easily obtained nasal biopsy may sometimes yield the diagnosis. The renal involvement is usually in the form of a necrotizing glomerulonephritis.

The disease typically affects middle aged men, and its formerly poor prognosis has been improved by corticosteroid and cyclophosphamide therapy.

Note:
Aspergillosis can cause prominent lung disease, but does not usually have renal involvement.
Polyarteritis nodosa is a possibility, but usually spares the lungs.
Renal cell carcinoma might cause lung nodules, but there is no evidence of tumor in the kidney.

Quiz In Between


Q. 4

In Wegener’s glomerulonephritis, the characteristic features seen is:

 A

Focal necrotizing glomerulonephritis

 B

Granulomas in the vessels wall

 C

Interstitial granulomas

 D

Nodular glomerulosclerosis

Q. 4

In Wegener’s glomerulonephritis, the characteristic features seen is:

 A

Focal necrotizing glomerulonephritis

 B

Granulomas in the vessels wall

 C

Interstitial granulomas

 D

Nodular glomerulosclerosis

Ans. B

Explanation:

Ans:B.)Granulomas in the vessels wall

  • Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology.
  • Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis in small- and medium-sized blood vessels.
  • Cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) directed against PR3 is most specific for GPA.

Q. 5

A 20 year old woman presents with hemoptysis, bilateral conductive deafness and palpable purpura on the legs. X ray chest shows a thin walled cavity in left lower zone. Investigations shows a leukocyte count of 12000/mm3, red cell casts in the urine and serum creatinine 3mg/dL. What is the most probable diagnosis?

 A

Henoch-Schonlein purpura

 B

Polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Disseminated tuberculosis

Q. 5

A 20 year old woman presents with hemoptysis, bilateral conductive deafness and palpable purpura on the legs. X ray chest shows a thin walled cavity in left lower zone. Investigations shows a leukocyte count of 12000/mm3, red cell casts in the urine and serum creatinine 3mg/dL. What is the most probable diagnosis?

 A

Henoch-Schonlein purpura

 B

Polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Disseminated tuberculosis

Ans. C

Explanation:

Wegener’s granulomatosis is characterized by granulomatous vasculitis of the upper and lower respiratory tracts together with glomerulonephritis.

Male-to-female ratio is 1:1. And the mean age of onset is 40 years.

Clinical features: Patients often present with paranasal sinus pain, purulent or bloody nasal discharge, with or without nasal mucosal ulceration, nasal septal perforation and serous otitis media.

Pulmonary involvement results in as cough, hemoptysis, dyspnea, and chest discomfort.

Due to renal disease patients develop proteinuria, hematuria, and red blood cell casts.

Ref: Harrison’s Internal Medicine, 18th Edition, Pages 2789, Chapter 326


Q. 6

Which of the following disease is more common in patients with alfa 1 antitrypsin deficiency?

 A

Good pasteurs syndrome

 B

Microscopic PAN

 C

Wegeners granulamatosis

 D

Churg –strauss syndrome

Q. 6

Which of the following disease is more common in patients with alfa 1 antitrypsin deficiency?

 A

Good pasteurs syndrome

 B

Microscopic PAN

 C

Wegeners granulamatosis

 D

Churg –strauss syndrome

Ans. C

Explanation:

Alfa 1 antitrypsin is an inhibitor of proteinase 3. Antiproteinase antibodies are seen in Wegeners granulamatosis. 

Ref: Harrisons Principles of Internal Medicine, 18th Edition, Page 2343

Quiz In Between


Q. 7

Wegener’s granulomatosis involve-

 A

Lung

 B

Liver

 C

Kidney

 D

a and c

Q. 7

Wegener’s granulomatosis involve-

 A

Lung

 B

Liver

 C

Kidney

 D

a and c

Ans. D

Explanation:

Ans. is ‘a’ i.e., Lung; ‘c’ i.e., Kidney

Wegener’s granulomatosis

o Wegener’s granulomatosis is a necrotizing and granulomatous vasculitis characterized by triad of ‑

  1. Acute necrotizing granulomas of upper and lower respiratory tract.
  2. Necrotizing granulomatous vasculitis affecting mainly lung, but other site as well.
  3. Renal disease focal proliferative crescentic glomerulonephritis. o Wegener’s granulomatosis is associated with c-ANCA. Clinical manifestations
  • Constitutional symptoms -4 Fever, malaise, fatigue, weight loss. o Respiratory system

         This is the first organ system to be involved.

         Upper respiratory tract —> Rhinitis, sinusitis, deafness, septal perforation, saddle nose, ulceration in nasopharynx.

          Lower respiratory tract —> Cough, chest pain, dysnea, hemoptysis, lung infiltrated, nodules and cavities.

  • Kidney

        Kidney involvement is the most dangerous manifestation.

          Heamaturia and non-nephrotic range proteinuria due to crescentic glomerulonephritis.

o Eye                                                  —>     Conjuctivitis, episcleritis, uveitis, keratitis, orbital pseudotumor, necrotizing scleritis.

o Joint                                                 —>         Migratory arthritis

o Skin                                                  —>         Palpable purpura

o GET                                                  —>         Mesentric vasculitis and splenic involvement.

o Peripheral nervous system           –>           Mononeuritis multiplex


Q. 8

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. 8

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.


Q. 9

In Wegeners glomerulonephritis characteristic feature seen in –

 A

Granuloma in the vessel wall

 B

Focal necrotizing glomerulonephritis

 C

Nodular glomerulosclerosis

 D

a and b

Q. 9

In Wegeners glomerulonephritis characteristic feature seen in –

 A

Granuloma in the vessel wall

 B

Focal necrotizing glomerulonephritis

 C

Nodular glomerulosclerosis

 D

a and b

Ans. D

Explanation:

Ans. is ‘b > a’ i.e., Focal necrotizing glomerulonephritis > Granuloma in the vessel wall

“Wegener’s granulomatosis is characterized by necrotizing granulomatous inflammation, small & medium – sized

vasculitis, and focal necrotizing glomerulonephritis, often with crescent formation”.

Text book of rheumatology vol 1-1942

o Wegener’s granulomatosis is primarily a necrotizing vasculitis with granulomas formation in vessel wall —> necrotizing granulomatous vasculitis.

o Granulomas in Wegener’s granulomatosis are seen only in lungs and respiratory tract. Granuloma formation is rarely seen in other organs. Other organs, only demonstrate necrotizing vasculitis

 

Quiz In Between


Q. 10

Which is not a characteristic of wegeners granulomatosis –

 A

Granuloma in vessel wall

 B

Focal necrotising glomerulonephritis

 C

Positive for c ANCA

 D

Involves large vessels

Q. 10

Which is not a characteristic of wegeners granulomatosis –

 A

Granuloma in vessel wall

 B

Focal necrotising glomerulonephritis

 C

Positive for c ANCA

 D

Involves large vessels

Ans. D

Explanation:

Ans. is ‘d’ i.e., Involves large vessels

Wegeners granulomatosis involves small vessels (arteriole, venule, capillary).


Q. 11

In Wegener’s granulomatosis cytoplasmic anti neutrophilic antibodies are directed against –

 A

Proteinase 1

 B

Proteinase 2

 C

Proteinase 3

 D

Proteinase 4

Q. 11

In Wegener’s granulomatosis cytoplasmic anti neutrophilic antibodies are directed against –

 A

Proteinase 1

 B

Proteinase 2

 C

Proteinase 3

 D

Proteinase 4

Ans. C

Explanation:

Ans. is ‘c’ i.e., Proteinase 3


Q. 12

RPGN caused by –

 A

FSGS

 B

Wegener’s granulomatosis

 C

Good pasteur’s syndrome

 D

b and c

Q. 12

RPGN caused by –

 A

FSGS

 B

Wegener’s granulomatosis

 C

Good pasteur’s syndrome

 D

b and c

Ans. D

Explanation:

Ans. is ‘b’ i.e., Wegener granulomatosis; ‘c’ i.e., Good pasture syndrome

Quiz In Between


Q. 13

Cresentic glomerular deposits are seen in

 A

Wegener’s granulomatosis

 B

Polyarteritis nodosa

 C

Thrombangitis obliterans

 D

All of the above

Q. 13

Cresentic glomerular deposits are seen in

 A

Wegener’s granulomatosis

 B

Polyarteritis nodosa

 C

Thrombangitis obliterans

 D

All of the above

Ans. A

Explanation:

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

Wegener’s granulomatosis causes RPGN, which is characterized by the presence of cresents in the glomeruli.


Q. 14

C-ANCA is associated with:

 A

Wegener’s Granulomatosis

 B

Microscopic Polyangitis

 C

Churg- Strauss Syndrome

 D

Polyarteritis Nodosa (PAN)

Q. 14

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. 15

A person with involvement of upper respiratory tract, lungs and kidney shows evidence of granulomas on histo pathology. The most likely diaglosis is:

 A

Wegener’s Granulomatosis

 B

Goodpasture Syndrome

 C

Tuberculosis

 D

Sarcoidosis

Q. 15

A person with involvement of upper respiratory tract, lungs and kidney shows evidence of granulomas on histo pathology. The most likely diaglosis is:

 A

Wegener’s Granulomatosis

 B

Goodpasture Syndrome

 C

Tuberculosis

 D

Sarcoidosis

Ans. A

Explanation:

Answer is A (Wegener’s Granulomatosis)

The charachteristic triad of vasculitis involving the upper respiratory tract, the lower respiratory tract (Lungs) and kidney along with granuloma formation on histopatholgy suggests a diagnosis of Wegener’s granulomatosis.

Quiz In Between


Q. 16

A 20 year old woman presents with bilateral conductive deafness, palpable purpura on the legs and hemoptysis. Radiograph of the chest shows a thin-walled cavity in left lower zone. Investigations reveal total leukocyte count 12000/mm3, red cell casts in the urine and serum creatinine 3mg/dL. What is the most probable diagnosis?

 A

Henoch-Schonlein purpura

 B

Polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Disseminated tuberculosis

Q. 16

A 20 year old woman presents with bilateral conductive deafness, palpable purpura on the legs and hemoptysis. Radiograph of the chest shows a thin-walled cavity in left lower zone. Investigations reveal total leukocyte count 12000/mm3, red cell casts in the urine and serum creatinine 3mg/dL. What is the most probable diagnosis?

 A

Henoch-Schonlein purpura

 B

Polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Disseminated tuberculosis

Ans. C

Explanation:

Answer is C (Wegener’s granulomatosis)

The patient in question has been depicted to have one manifestation each of the classical triad as mentioned below along with skin lesion in the _Pm of palpable purpura and hence the answer is clearly Wegener’s granulomatosis.

Wegener’s granulomatosis in its classical form presents as characteristic triad of ‑

– Upper respiratory infection (ENT manifestations): includes otitis media (conductive deafness)

– Lower respiratory tract infection (Lung): includes haemoptysis and radiological cavity.

– Renal involvement: includes red cell casts and raised serum creatinine.

Skin lesions are seen in 46% of patients and may take the form of palpable purpura.

Wegener’s granulomatosis: Review

 

Wegener’s granulomatosis is a distinct clinicopathological entity characterized ‑

Clinically

Triad of

 

Upper respiratory tract manifestation (ENT)

Lower respiratory tract° manifestations (Lung)

Renal manifestations’

 

–     Otitis media (conductive deafness)

–     Paranasal sinus pain & drainage

–     Septal perforationQ

Other manifestations

–     Cough, haemoptysis, dyspneaQ

–     Multiple bilateral cavitatory nodular

infiltrates

–     Rapidly progressive rena failureQ

–     Crescentric glomerulonephritisQ

FeverQ, skin manifestationQ in the form of papules, vesicles, palpable purpura etc eye manifestationsQ, joint

manifestationsQ cardiac manifestations° and nervous system manifestationsQ.

 

Pathologically by

•    Necrotizing vasculitis of smalls’ arteries and veins

•    Intravascular and extravascular granuloma formation

Q Granulomas contain multiple well defined multinucleated giant cells

Bronchoalveolar lavage fluid contains high percentage of neutrophils compared to other granulomatous diseases which contain increased number of Lymphocytes

Q c-ANCA is the predominant ANCA in the disease

Characteristic laboratory findings include:

I .   Positive c-ANCA0

2.   Elevated ESR (mild elevation)

3.   RF° (often elevated)

4.   Hypergammaglobunemia (particularly IgA class)Q

•    Hypocomplementemia is not seen despite presence of circulating immune complexes”

Treatment of choice is: cyclophosphamide Q


Q. 17

A 25 years old female develops serous otitis media of left ear with cough and occasional hemoptysis and hematuria and epistaxis for one and half months her hemoglobin is 7 gm. B.P is > 170/100, proteinuria +++, RA – ve and ANCA – ve, the likely cause is‑

 A

Wegener’s granulomatosis

 B

Rheumiatoid arthritis

 C

Rapidly proliferative glomerulonephritis

 D

Goodpasteur’s syndrome

Q. 17

A 25 years old female develops serous otitis media of left ear with cough and occasional hemoptysis and hematuria and epistaxis for one and half months her hemoglobin is 7 gm. B.P is > 170/100, proteinuria +++, RA – ve and ANCA – ve, the likely cause is‑

 A

Wegener’s granulomatosis

 B

Rheumiatoid arthritis

 C

Rapidly proliferative glomerulonephritis

 D

Goodpasteur’s syndrome

Ans. A

Explanation:

Answer is A>D (Wegener’s granulumatosis > Goodpasture’s Syndrome);

The patient in question is presenting with characteristic clinical triad of Wegener’s granulomatosis, namely, Vasculitis of upper respiratory tract (serous otitis media, epistaxis), Vasculitis of lower respiratory tract (cough and hemoptysis) and vasculitis of the kidney (proteinuria)

Although positive ANCA assays are often instrumental in suggesting the diagnosis of WG, Negative ANCA assays do not preclude the diagnosis, 10% of patients with disseminated WG and upto 30% of patients with limited WG may be negative for ANCA

The clinical picture is classical of Wegner’s Granulomatosis. However, the absence of ANCA confuses the diagnosis. Nevertheless the presence or absence of ANCA should be adjunctive and in the presence of a classical clinical picture of WG, tissue diagnosis (biopsy) should be performed to confirm the diagnosis.

Definitive diagnosis of WG is established by tissue biopsy and not by presence or absence of ANCA

Pulmonary + Renal Syndrome
Pulmonary (Hemoptysis) + Renal (Proteinuria) manifestations

Wegener’s granulomatosis

Classical Clinical presentation is one of a young Male/Female (male: female=1:1) presenting with upper respiratory tract features (Otitis media, Epistaxis) along with hemoptysis and acute glomerulonephritis+

ANCA is usually positive and suggests the diagnosis

Negative ANCA does not preclude the diagnosis. In the presence of classical clinical picture a provisional diagnosis of WG should be made on clinical ground and confirmed/ excluded by tissue biopsy

The patient in question is presenting with classical clinical triad of Wegener’s granulomatosis. Although ANCA is negative a negative ANCA does not preclude the diagnosis of WG. Based on the strong clinical suspicion, WG should the single best initial provisional diagnosis and tissue biopsy should be performed.

Goodpasture’s syndrome

 

Classical clinical presentation is one of a young male smoker (male female=3-4:1) presenting with hemoptysis and signs of acute glomerulonephritis

 

Although disease may be preceeded by an upper respiratory tract infection, upper respiratory tract involvement features like otitis media and epistaxis are not characterstic of good pasture’s syndrome

 

ANCA is usually negative

 

Diagnosis is made by detecting serum levels of Anti GBM antibodies

 

The patient in question is not presenting with classical clinical features of good pasture’s syndrome and serum levels of Anti GBM antibodies have not been provided in the question. These make a diagnosis of Good pasture’s syndrome less likely.




Q. 18

Wegener’s granulomatosis does not affect:

 A

Kidney

 B

Lungs

 C

Eye

 D

Liver

Q. 18

Wegener’s granulomatosis does not affect:

 A

Kidney

 B

Lungs

 C

Eye

 D

Liver

Ans. D

Explanation:

Answer is D (Liver)

Granulomatous vasculitis involving the Liver is rare in Wegener’s granulomatosis. Wegener’s granulomotosis does not typically involve the liver.

Liver function tests may be observed to be abnormal in Wegener’s granulomatosis. These most commonly reflect inflammation rather than specific liver involvement. Granulomata and hepatic blood vessel involvement (Liver involvement) may be seen, but this is rare.’ – Vasculitis in Clinical Practice

Quiz In Between


Q. 19

Which of the following is more frequently seen in Churg Strauss Syndrome in comparison to Wegener’s Granulomatosis

 A

Renal involvement

 B

Lower Respiratory Tract involvement

 C

Eye involvement

 D

Upper Respiratory Tract involvement

Q. 19

Which of the following is more frequently seen in Churg Strauss Syndrome in comparison to Wegener’s Granulomatosis

 A

Renal involvement

 B

Lower Respiratory Tract involvement

 C

Eye involvement

 D

Upper Respiratory Tract involvement

Ans. B

Explanation:

Answer is B (Lower Respiratory Tract involvement):

Lower Respiratory Tract involvement is typically more common in Churg Strauss Syndrome in comparison to Wegener’s Granulomatosis.

Organs involved in small vessels vasculitis:

Organ involvement throughout disease course

Wegner’s granulomatosis

Microscopic polyangitis

Churg-Strauss syndrome

Upper respiratory tract

80%

Uncommon

55%

Lower respiratory tract

70%

30%

100%

Eye

46%

10%

6%

Kidney

80%

90%

33%

Peripheral nervous system

35%

20%

67%

Gastrointestinal system

5%

5%

17%

Heart

Uncommon

Uncommon

28%

Skin

50%

25%

44%

Constitutional symptoms

95%

90%

94%


Q. 20

Ocular manifestations of Wegener’s granulo­matosis include all of the following except

 A

Proptosis

 B

Nasolacrimal duct obstruction

 C

Necrotizing scleritis

 D

Internal ophthalmoplegia

Q. 20

Ocular manifestations of Wegener’s granulo­matosis include all of the following except

 A

Proptosis

 B

Nasolacrimal duct obstruction

 C

Necrotizing scleritis

 D

Internal ophthalmoplegia

Ans. D

Explanation:

Ans. Internal ophthalmoplegia


Q. 21

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. 21

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.

Quiz In Between


Q. 22

Which of the following is treatment of choice for Wegener’s granulomatosis:

 A

Antibiotics

 B

Steroids

 C

Analgesics

 D

Antiviral therapy

Q. 22

Which of the following is treatment of choice for Wegener’s granulomatosis:

 A

Antibiotics

 B

Steroids

 C

Analgesics

 D

Antiviral therapy

Ans. B

Explanation:

Ans. B: Steroids

Management of systemic vasculitis (which includes Wegener’s granulomatosis) is with high dose corticosteroids and immunosuppressives

Wegener’s granulomatosis:

  • Necrotizing vasculitis of small arteries and veins together with granuloma formation that can be either intravascular or extravascular

Lung involvement:

– Bilateral nodular cavitary infiltrates,

– demonstrate necrotizing Granulomatous vasculitis

– Renal biopsy shows lesion that of pauci-immune necrotizing and crescentic GN


Q. 23

CANCA is associated with ‑

 A

Wegener’s granulomatosis

 B

Microscopic polyangitis

 C

Chrug strauss syndrome

 D

Good pasture syndrome

Q. 23

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


Q. 24

Anti-neutrophil cytoplasmic antibodies (ANCA) is seen in

 A

Wegener’s Granulomatosis

 B

Diabetes mellitusRheumatoid arthritis

 C

Churg

 D

Strauss syndrome

Q. 24

Anti-neutrophil cytoplasmic antibodies (ANCA) is seen in

 A

Wegener’s Granulomatosis

 B

Diabetes mellitusRheumatoid arthritis

 C

Churg

 D

Strauss syndrome

Ans. A

Explanation:

Ans. a. Wegener’s


Q. 25

ANCA positive vasculitis 

 A

Henoch schonlein purpura

 B

Behcet’s syndrome

 C

Wegener’s granulomatosis

 D

Non

Q. 25

ANCA positive vasculitis 

 A

Henoch schonlein purpura

 B

Behcet’s syndrome

 C

Wegener’s granulomatosis

 D

Non

Ans. C

Explanation:

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

Quiz In Between



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