Polyarteritis Nodosa

POLYARTERITIS NODOSA

Q. 1 A 50-year-old drug abuser presents with fever and weight loss. Exam shows hypertension, nodular skin rash, and peripheral neuropathy. ESR is 100 mm/L, and RBC casts are seen on urinalysis. (SELECT 1 DIAGNOSIS)
 A Behcet syndrome
 B Ankylosing spondylitis
 C Polymyalgia rheumatic
 D Polyarteritis nodosa
Q. 1 A 50-year-old drug abuser presents with fever and weight loss. Exam shows hypertension, nodular skin rash, and peripheral neuropathy. ESR is 100 mm/L, and RBC casts are seen on urinalysis. (SELECT 1 DIAGNOSIS)
 A Behcet syndrome
 B Ankylosing spondylitis
 C Polymyalgia rheumatic
 D Polyarteritis nodosa
Ans. D

Explanation:

Behcet syndrome is a multisystem disorder that usually presents with recurrent oral and genital ulcers. Onefourth of patients develop superficial or deep vein thrombophlebitis. Iritis, uveitis, and nondeforming arthritis may also occur. The 50-year-old drug abuser also has a multisystem disease, including systemic complaints, hypertension, skin lesions, neuropathy, and an abnormal urine sediment. This complex suggests a vasculitis, particularly polyarteritis nodosa. The disease is a necrotizing vasculitis of small and medium muscular arteries. The pathology of the kidney includes an arteritis and, in some cases, a glomerulitis. Nodular skin lesions show vasculitis on biopsy. The 19-year-old with low back pain, morning stiffness, and eye pain has complaints that suggest ankylosing spondylitis. This is an inflammatory disorder that affects the axial skeleton. It is an autoimmune disorder that has a close association with HLA-B27 histocompatibility antigen. Anterior uveitis is the most common extraarticular complaint. Aortic regurgitation occurs in a few percent of patients.

The elderly male presents with nonspecific joint complaints typical of polymyalgia rheumatica. The high erythrocyte sedimentation rate is characteristic. The transient loss of vision suggests concomitant temporal arteritis, an important association seen particularly.


Q. 2 A 50-year-old drug abuser presents with fever and weight loss. Exam shows hypertension, nodular skin rash, and peripheral neuropathy. ESR  is 100  mm/L, and RBC casts are seen on urinalysis. The most likely condition is:
 A Reiter syndrome
 B Drug-induced lupus erythematosus
 C Polyarteritis nodosa 
 D Behçet syndrome
Q. 2 A 50-year-old drug abuser presents with fever and weight loss. Exam shows hypertension, nodular skin rash, and peripheral neuropathy. ESR  is 100  mm/L, and RBC casts are seen on urinalysis. The most likely condition is:
 A Reiter syndrome
 B Drug-induced lupus erythematosus
 C Polyarteritis nodosa 
 D Behçet syndrome
Ans. C

Explanation:

The 50-year-old gentleman is a drug abuser and also has a multisystem disease, including systemic complaints, hypertension, skin lesions, neuropathy, and an abnormal urine sediment. This complex suggests a vasculitis, particularly polyarteritis nodosa. 20 to 30% of patients have hepatitis B antigenemia. The disease is  a  necrotizing vasculitis of  small and  medium muscular arteries. The pathology of the kidney includes an arteritis and, in some cases, a glomerulitis. Nodular skin lesions show vasculitis on biopsy.


Q. 3 Palpable purpura are seen in all EXCEPT:
 A

Giant cell arteritis

 B

HSP

 C Wegner granulomatosis
 D Polyarteritis nodosa
Q. 3 Palpable purpura are seen in all EXCEPT:
 A

Giant cell arteritis

 B

HSP

 C Wegner granulomatosis
 D Polyarteritis nodosa
Ans. A

Explanation:

Giant cell arteritis

REF: Harrison’s 18′ ed chapter 53 table 54-16

“Giant cell arteritis being a large vessel vasculitis does not cause any purpura”

CAUSES OF PURPURA:

 

Palpable

Non palpable

Primary cutaneous

 

Trauma

disorder

 

Solar purpura

 

 

Steroid purpura

 

 

Capillaritis

 

 

Livedoid vasculopathy

Systemic disorder

Vasculitis

Vascular fragility

 

•          Cutaneous small-vessel

•          Amyloidosis

 

vasculitis (Henoch-Schänlein

•          Ehlers-Danlos syndrome

 

purpura, Acute hemorrhagic

•          Scurvy

 

edema of infancy, Urticarial

Thrombi

 

vasculitis, Cryoglobulinemic

•          DIC

 

vasculitis, Erythema elevatum

•          Monoclonal cryoglobulinemia

 

diutinum, Granuloma faciale)

•          TTP

 

•          Polyarteritis nodosa

•          Warfarin reaction

 

Emboli

Emboli

 

•          Acute meningococcemia

•           Cholesterol

 

•           Disseminated gonococcal

•           Fat

 

infection

Possible immune complex

 

•           Rocky Mountain spotted fever

•       Gardner-Diamond syndrome

 

•           Ecthyma gangrenosum

(autoerythrocyte sensitivity)

 

 

•           Waldenstrom’s

hypergammaglobulinemic purpura

 

 

Clotting disturbances

 

 

•           ITP

 

 

•           Abnormal platelet function

 

 

•           Clotting factor defects

 


Q. 4

Fibrinoid necrosis may be observed in all of the following, EXCEPT:

 A

Malignant hypertension

 B

Polyarteritis nodosa

 C

Diabetic glomerulosclerosis

 D

Aschoff’s nodule

Q. 4

Fibrinoid necrosis may be observed in all of the following, EXCEPT:

 A

Malignant hypertension

 B

Polyarteritis nodosa

 C

Diabetic glomerulosclerosis

 D

Aschoff’s nodule

Ans. C

Explanation:

Fibrinoid necrosis is a type of connective tissue necrosis seen particularly in autoimmune diseases (eg, rheumatic fever, polyarteritis nodosa, and systemic lupus erythematosus).

Collagen and smooth muscle in the media of blood vessels are especially involved.

Fibrinoid necrosis of arterioles also occurs in accelerated (malignant) hypertension.

Fibrinoid necrosis is characterized by loss of normal structure and replacement by a homogeneous, bright pink-staining necrotic material that resembles fibrin microscopically.
Areas of fibrinoid necrosis contain various amounts of immunoglobulins and complement, albumin, breakdown products of collagen, and fibrin.
 
Also Know:
 
Fat Necrosis:
Fat necrosis most characteristically occurs in acute pancreatitis when pancreatic enzymes are liberated from the ducts into surrounding tissue. Pancreatic lipase acts on the triglycerides in fat cells, breaking these down into glycerol and fatty acids, which complex with plasma calcium ions to form calcium soaps. 
 
Liquefactive Necrosis:
Liquefaction of necrotic cells results when lysosomal enzymes released by the necrotic cells cause rapid liquefaction. Lysis of a cell as a result of the action of its own enzymes is autolysis. Liquefactive necrosis is typically seen in the brain following ischemia.
 
Coagulative Necrosis:
In this type of necrosis, the necrotic cell retains its cellular outline, often for several days. The cell, devoid of its nucleus, appears as a mass of coagulated, pink-staining, homogeneous cytoplasm.
 
Coagulative necrosis typically occurs in solid organs, such as the kidney, heart (myocardium), and adrenal gland, usually as a result of deficient blood supply and anoxia. It is also seen with other types of injury, eg, coagulative necrosis of liver cells due to viruses or toxic chemicals, and coagulative necrosis of skin in burns.
 
Ref: Chandrasoma P., Taylor C.R. (1998). Chapter 1. Cell Degeneration & Necrosis. In P. Chandrasoma, C.R. Taylor (Eds), Concise Pathology, 3e

Q. 5

A 35 year old man presents to his doctor with loss of appetite, nausea and vomiting, and fatigue. Laboratory examination confirms the diagnosis of hepatitis B, and the man becomes icteric 2 weeks later. This patient may also be particularly vulnerable to the development of which of the following disorders?

 A

Berry aneurysm

 B

Coronary artery aneurysm

 C

Dissecting aneurysm

 D

Polyarteritis nodosa

Q. 5

A 35 year old man presents to his doctor with loss of appetite, nausea and vomiting, and fatigue. Laboratory examination confirms the diagnosis of hepatitis B, and the man becomes icteric 2 weeks later. This patient may also be particularly vulnerable to the development of which of the following disorders?

 A

Berry aneurysm

 B

Coronary artery aneurysm

 C

Dissecting aneurysm

 D

Polyarteritis nodosa

Ans. D

Explanation:

Thirty percent of patients with polyarteritis nodosa have hepatitis B antigenemia.
Polyarteritis is a systemic necrotizing vasculitis that can be difficult to diagnose, since the vascular involvement is typically widely scattered, and the specific symptoms depend on the specific vessels (small- to medium-sized arteries) involved.
Patients typically present with low-grade fever, weakness, and weight loss.
Abdominal pain, hematuria, renal failure, hypertension, and leukocytosis may occur.
The disease is frequently fatal if untreated.
Berry aneurysms are congenital saccular dilatations of vessels associated with adult polycystic kidney disease.
Coronary artery aneurysms are associated with Kawasaki’s disease in children.
Dissecting aneurysms are associated with cystic medial necrosis (seen in Marfan’s syndrome) and hypertension.
 
Ref: Wyatt C., Butterworth IV J.F., Moos P.J., Mackey D.C., Brown T.G. (2008). Chapter 9. Vascular Pathology. In C. Wyatt, J.F. Butterworth IV, P.J. Moos, D.C. Mackey, T.G. Brown (Eds), Pathology: The Big Picture.

 


Q. 6

FALSE statement about polyarteritis nodosa (PAN) is:

 A

It is a necrotizing vasculitis of small and medium-sized muscular arteries

 B

Involvement of the renal and visceral arteries is characteristic.

 C

PAN does not involve pulmonary arteries and bronchial vessels

 D

Granulomas, significant eosinophilia, and are not observed.

Q. 6

FALSE statement about polyarteritis nodosa (PAN) is:

 A

It is a necrotizing vasculitis of small and medium-sized muscular arteries

 B

Involvement of the renal and visceral arteries is characteristic.

 C

PAN does not involve pulmonary arteries and bronchial vessels

 D

Granulomas, significant eosinophilia, and are not observed.

Ans. C

Explanation:

Polyarteritis nodosa (PAN)

  • It is a multisystem, necrotizing vasculitis of small and medium-sized muscular arteries.
  • Involvement of the renal and visceral arteries is characteristic.
  • PAN does not involve pulmonary arteries, although bronchial vessels may be involved;
  • Granulomas, significant eosinophilia, and an allergic diathesis are not observed.

Ref: Harrisons principles of internal medicine, 18th edition, Page: 2794.


Q. 7

A 25 year old man presents to the emergency room with severe abdominal pain. Guaiac test on stool demonstrates occult blood. The patient is taken to exploratory laparotomy, at which a small area of infarcted small bowel is found and surgically removed. Histologic studies on the removed section of bowel demonstrate a recent thrombus occluding a small muscular artery. The adjacent vessel wall shows fibrinoid necrosis with a mixed inflammatory infiltrate containing neutrophils, eosinophils, and mononuclear cells. Which of the following is the most likely cause of the small bowel infarction?

 A

Atherosclerosis

 B

Cystic medial necrosis

 C

Mönckeberg’s arteriosclerosis

 D

Polyarteritis nodosa

Q. 7

A 25 year old man presents to the emergency room with severe abdominal pain. Guaiac test on stool demonstrates occult blood. The patient is taken to exploratory laparotomy, at which a small area of infarcted small bowel is found and surgically removed. Histologic studies on the removed section of bowel demonstrate a recent thrombus occluding a small muscular artery. The adjacent vessel wall shows fibrinoid necrosis with a mixed inflammatory infiltrate containing neutrophils, eosinophils, and mononuclear cells. Which of the following is the most likely cause of the small bowel infarction?

 A

Atherosclerosis

 B

Cystic medial necrosis

 C

Mönckeberg’s arteriosclerosis

 D

Polyarteritis nodosa

Ans. D

Explanation:

The lesion described is that of polyarteritis nodosa.

This condition is often considered to be the prototype for the vasculitides, and characteristically affects small or medium-sized muscular arteries, with a predilection for the gastrointestinal tract and kidney (causing hematuria, albuminuria, or renal failure).

Patients are typically young adult males, although both sexes and all ages may have the condition. Characteristically, vascular lesions of different stages may be present.

Corticosteroid and cyclophosphamide therapy has improved a formerly poor prognosis.
 
Atherosclerosis can cause bowel infarction, but the histologic description would be that of a plaque.
 
Cystic medial necrosis predisposes for dissecting aortic aneurysm.
 
Mönckeberg’s arteriosclerosis is a benign condition characterized by ring-like calcifications of vessel walls.
 
Ref: Lin P.H., Kougias P., Bechara C., Cagiannos C., Huynh T.T., Chen C.J. (2010). Chapter 23. Arterial Disease. In F.C. Brunicardi, D.K. Andersen, T.R. Billiar, D.L. Dunn, J.G. Hunter, J.B. Matthews, R.E. Pollock (Eds), Schwartz’s Principles of Surgery, 9e.

Q. 8

Which of the following conditions poses the greatest risk for development of pre-eclampsia?

 A

Chronic glomerulonephritis

 B

Polycystic kidney disease

 C

Renal artery stenosis

 D

Polyarteritis nodosa

Q. 8

Which of the following conditions poses the greatest risk for development of pre-eclampsia?

 A

Chronic glomerulonephritis

 B

Polycystic kidney disease

 C

Renal artery stenosis

 D

Polyarteritis nodosa

Ans. D

Explanation:

The risk of developing pre–eclampsia is only mild in chronic glomerulo nephritis and polycystic kidney disease.

The risk is mild to moderate in Renal artery stenosis and severe in Polyarteritis nodosa.

Ref: Karla Philip, 3rd Edition, Page 302.

 


Q. 9

An 18 year old boy presents with digital gangrene of the third and fourth fingers over the last 2 weeks. On examination the blood pressure is 170/110 mm of Hg and all peripheral pulses were palpable. Blood and urine examinations were unremarkable. ANA, Anti-dsDNA and ANCA were negative. Which of the following represents the most likely diagnosis?

 A

Wegener’s granulomatosis

 B

Polyarteritis nodosa

 C

Takayasu’s arteritis

 D

Systemic lupus erythematosis (SLE)

Q. 9

An 18 year old boy presents with digital gangrene of the third and fourth fingers over the last 2 weeks. On examination the blood pressure is 170/110 mm of Hg and all peripheral pulses were palpable. Blood and urine examinations were unremarkable. ANA, Anti-dsDNA and ANCA were negative. Which of the following represents the most likely diagnosis?

 A

Wegener’s granulomatosis

 B

Polyarteritis nodosa

 C

Takayasu’s arteritis

 D

Systemic lupus erythematosis (SLE)

Ans. B

Explanation:

Patient is showing features of PAN. It  is a multisystem, necrotizing vasculitis of small and medium-sized muscular arteries mainly involving the renal and visceral arteries.

It does not involve pulmonary arteries, although bronchial vessels may be involved.

Clinical features: Patients often present with nonspecific symptoms such as fever, weight loss, and malaise and weakness. Hypertensions, arthritis, peripheral neuropathy, cutaneous infarcts, Raynaud’s phenomenon are complaints related to specific organ system involvement. 

Investigations: There are no diagnostic serologic tests for PAN. Anemia of chronic disease, neutrophilic leukocytosis, and an elevated ESR is almost always present.

Hypergammaglobulinemia may be present, and all patients should be screened for hepatitis B.

Antibodies against myeloperoxidase or proteinase-3 (ANCA) are rarely found in patients with PAN.

Ref: Harrison’s Internal Medicine, 18th Edition, Chapter 326, Pages 2726-2727, 2789, 2794, 2796


Q. 10

A 30 years old male patient presents with weakness of right upper and both lower limbs since 4 months. He developed digital infarcts involving 2nd and 3rd fingers on right side and 5th finger on left side. On examination, BP was 160/140 mm Hg, all peripheral pulses were palpable and there was asymmetrical neuropathy. Investigations showed a Hb 12 gm, TLC – 12000 Cu mm. Platelets 4,30.000. ESR – 49 mm. Urine examination showed proteinuria and RBC -10-15/hpf with no casts. Which of the following is the most likely diagnosis?

 A

Polyarteritis nodosa

 B

Mixed cryoglobulinemia

 C

Wegener’s granulomatosis

 D

Systemic lupus erythematosus

Q. 10

A 30 years old male patient presents with weakness of right upper and both lower limbs since 4 months. He developed digital infarcts involving 2nd and 3rd fingers on right side and 5th finger on left side. On examination, BP was 160/140 mm Hg, all peripheral pulses were palpable and there was asymmetrical neuropathy. Investigations showed a Hb 12 gm, TLC – 12000 Cu mm. Platelets 4,30.000. ESR – 49 mm. Urine examination showed proteinuria and RBC -10-15/hpf with no casts. Which of the following is the most likely diagnosis?

 A

Polyarteritis nodosa

 B

Mixed cryoglobulinemia

 C

Wegener’s granulomatosis

 D

Systemic lupus erythematosus

Ans. A

Explanation:

Patient in the question is suffering from hypertension, digital infarcts in presence of palpable peripheral pulses and urine examination showing proteinuria and hematuria.

All of these are features of polyarteritis nodosa a necrotizing vasculitis of small and medium-sized muscular arteries with characteristic involvement of the renal and visceral arteries.

It does not involve pulmonary arteries, sometimes bronchial vessels may be involved.

Clinical features: Patients presents with fever, weight loss, and malaise, headache, abdominal pain and myalgias. Renal involvement manifests as hypertension, renal insufficiency, or hemorrhage due to microaneurysms.

Investigations: CBC shows neutrophilic leucocytosis, anemia of chronic disease and an elevated ESR. Antibodies against myeloperoxidase or proteinase-3 (ANCA) is rarely found. Urine shows evidence of proteinuria and hematuria.

Diagnosis is made by the biopsy of the organ involved, which shows evidence of vasculitis.

Ref: Harrison’s Internal Medicine, 18th Edition, Chapter 283; Current Rheumatology Diagnosis and Treatment By John B. Imboden, 2nd Edition, Chapter 33


Q. 11

All of the following conditions are associated with palpable purpura, EXCEPT:

 A

Giant cell arteritis

 B

Henoch Schonlein Purpura

 C

Wegner granulomatosis

 D

Polyarteritis nodosa

Q. 11

All of the following conditions are associated with palpable purpura, EXCEPT:

 A

Giant cell arteritis

 B

Henoch Schonlein Purpura

 C

Wegner granulomatosis

 D

Polyarteritis nodosa

Ans. A

Explanation:

Differential diagnosis of palpable purpura includes Henoch-Schonlein purpura (HSP), cryoglobulinemia or cryofibrinogenemia, polyarteritis nodosa (PAN), Wegener’s granulomatosis, infections, and collagen vascular disorders.

Giant cell arteritis being a large vessel vasculitis does not cause any purpura.


Q. 12

In polyarteritis nodosa, aneurysms are seen in all, except:

 A

Kidney

 B

Lung

 C

Liver

 D

Pancreas

Q. 12

In polyarteritis nodosa, aneurysms are seen in all, except:

 A

Kidney

 B

Lung

 C

Liver

 D

Pancreas

Ans. B

Explanation:

Answer is B (Lung):

‘PAN (classic PAN) does not involve pulmonary arteries although bronchial vessels may be involved. Aneurysmal dilatation npto 1 cm in size along the involved arteries is characteristic of PAN.r-Harrison 16th/2007.

Classical PAN: Review

Classical PAN is a multisystem necrotizing vasculitis of small & medium sized muscular arteries in which involvement of renal & visceral arteries is characteristic

Clinical Manifestations Related to Organ System Involvement in Classic Polyarteritis Nodosa

Renal

Hypertension, Renal failure

Musculoskeletal

Arthritis, arthralg a, myalgia

Peripheral nervous system

Peripheral neuropathy, mononeuritis multiplex

Gastrointestinal tract

Abdominal pain, nausea and vomiting, bleeding, bowel infarction and perforation,

cholecystitis, hepatic infarction, pancreatic infarction

Skin

Rash, purpura, nodules, cutaneous infarcts, livedo reticularis, Raynaud phenomenon

Cardiac

Congestive heart failure, myocardial infarction, pericarditis

Genitourm!ig

Testicular, ovarian, or epididymal pain

Central nervous system

Cerebral vascular accident, altered mental status, seizure

Remember:

  • Classic PAN does not involve pulmonary arteries. Bronchial vessels may be involved but their involvement also, is an uncommon Jeature.Q
  • Renal involvement is characterized by arteritis without glomerulonephritis. Clinically renal involvement is in the form of renovascular hypertension is frequent. Renal failure may occur but it is uncommon and usually occurs late.Q

Q. 13

A 30 years old male patient presents with complaints of weakness in right upper and both lower limbs of last 4 months. He developed digital infarcts involving 2nd and 3rd fingers on right side and 5`h fingeron left side. On examination, BP was 160/140 mm Hg, all peripheral pulses were palpable and there was asymmetrical neuropathy. Investigations showed a

Hb 12 gm, TLC-12000 Cu mm, Platelets 4,30,000, ESR-49 mm. Urine examination showed proteinuria and RBC – 10-15/hpf with no casts. Which of the following is the most likely diagnosis?

 A

Polyarteritis nodosa

 B

Systemic lupus erythematosus

 C

Wegener’s granulomatosis

 D

Microscopic polyangitis

Q. 13

A 30 years old male patient presents with complaints of weakness in right upper and both lower limbs of last 4 months. He developed digital infarcts involving 2nd and 3rd fingers on right side and 5`h fingeron left side. On examination, BP was 160/140 mm Hg, all peripheral pulses were palpable and there was asymmetrical neuropathy. Investigations showed a

Hb 12 gm, TLC-12000 Cu mm, Platelets 4,30,000, ESR-49 mm. Urine examination showed proteinuria and RBC – 10-15/hpf with no casts. Which of the following is the most likely diagnosis?

 A

Polyarteritis nodosa

 B

Systemic lupus erythematosus

 C

Wegener’s granulomatosis

 D

Microscopic polyangitis

Ans. A

Explanation:

Answer is A (Polyarteritis nodosa):

Renal disease manifesting as hypertension without RBC cast in urine (No glomerulonephritis) is characteristic of polyarteritis Nodosa
Digital gangrene is usually associated with vasculitis of medium sized vessels. Amongst options provided PAN is a medium vessel vasculitis and presence of digital ischaemia is more characteristic of PAN than MPA or WG. Digital gangrene may however be seen (less frequent) in cases of MPA and WG.


Q. 14

An 18 year old boy presents with digital gangrene in 3rd & 4th fingers for last 2 weeks. On examination the BP is 170/110 mm of Hg and all peripheral pulses were palpable. Blood & Urine examination were unremarkable. Antinuclear antibody, Antibody to ds DNA & DNA & ANCA were negative. Most likely diagnosis is:

 A

Henoch-Schonlein purpura

 B

Polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Disseminated tuberculosis

Q. 14

An 18 year old boy presents with digital gangrene in 3rd & 4th fingers for last 2 weeks. On examination the BP is 170/110 mm of Hg and all peripheral pulses were palpable. Blood & Urine examination were unremarkable. Antinuclear antibody, Antibody to ds DNA & DNA & ANCA were negative. Most likely diagnosis is:

 A

Henoch-Schonlein purpura

 B

Polyarteritis nodosa

 C

Wegener’s granulomatosis

 D

Disseminated tuberculosis

Ans. B

Explanation:

Answer is B (Polyarteritis Nodosa)

The clinical presentation of the patient in question is consistent with a diagnosis of Polyarteritis Nodosa.

The clinical presentation of patient in question gives us the following ‘leads’ in approaching the diagnosis.

  • Digital gangrene in the setting of normal peripheral pulses.
  • Presence of hypertension
  • Negative antinuclear antibodies
  • Negative anti dsDNA
  • Negative ANCA

Polvarteritis Nodosa: PAN

 

  • Classic Polyarteritis nodosa is characterized by necrotizing inflammation of medium sized muscular arteries.
  • In classic Polyarteritis Nodosa, skin findings include livido reticularis, subcutaneous nodules and skin ulcers. Digital gangrene() is not an unusual occurance — CMDT/ 2004/813
  • Involvement of renal arteries leads to renin mediated hypertension().
  • Patients with classic PAN are ANCA negative — CMDT 2004/814
  • Positive ANCA (pANCA) are found in low percentage (< 20%) of patients with classical PAN- Harrison [ANCAs that produce a
    p-ANCA pattern on immunofluorescence testing are frequently associated with microscopic polyangitis and not classical PAN.
  • Polyarteritis nodosa is a term given to necrotizing arteritis of medium sized vessels. CMDT 2004
  • Microscopic polyangitis may involve medium sized as well as small blood vessels and its spectrum overlaps that of PAN.
  • The clinical presentation of patient in question is consistent with a diagnosis of PAN.

 

Takayasu’s disease is also known as `pulseless disease’ and absence of peripheral pulses is a clinical hallmark of disease. The presence of all peripheral pulses makes this diagnosis unlikely.

 

SLE: Absence of ANA and Anti ds DNA almost rules out the possibility of SLE as a probable diagnosis.

 

Wegener’s granulomatosis is a necrotizing granulomatous vasculitis of small arteries and skin manifestations does not include digital gangrene.

 

Antineutrophil cytoplasmic antibodies (ANCA) occur is about 90% of patients with active Wegner’s granulomatosis. Absence of ANCA in blood strongly points against a diagnosis of Wegener’s granulomatosis



Q. 15

A 30 year old male presents with numbness of both lower limbs and right upper limb. Examination reveals pulse 88/minutes and BPI 160/110 mm of Hg. He also has digital gangrene involving right 2nd and 3rd finger, urine routine examination is unremarkable. Microscopic examination shows RBC’s. Hemogram and serum biochemistry is within normal limits. What is the most probable diagnosis?

 A

Systemic lupus erythematosus

 B

Polyarteritis nodosa

 C

Malignant hypertension

 D

Chugh-Strauss syndrome

Q. 15

A 30 year old male presents with numbness of both lower limbs and right upper limb. Examination reveals pulse 88/minutes and BPI 160/110 mm of Hg. He also has digital gangrene involving right 2nd and 3rd finger, urine routine examination is unremarkable. Microscopic examination shows RBC’s. Hemogram and serum biochemistry is within normal limits. What is the most probable diagnosis?

 A

Systemic lupus erythematosus

 B

Polyarteritis nodosa

 C

Malignant hypertension

 D

Chugh-Strauss syndrome

Ans. B

Explanation:

Ans. is B i.e. (Polyarteritis nodosa)

Polyarteritis nodosa is the single best answer amongst the options provided.

  • Numbness of multiple limbs indicates peripheral neuropathy / mononeuritis multiplex. `PAN is amongst the forms of vasculitis most commonly associated with vasculitic neuropathy’- CMDT.
  • Digital zangrene forms part of the typical skin findings in PAN.

`Typical skin findings in PAN include Livido Reticularis, subcutaneous nodules and skin ulcers. Digital gangrene is not uncommon’ – Harrison

  • Hypertension (BP = 160/110mmHg) is a common manifestation of renal involvement in PAN.

`Involvement of renal arteries leads to a renin mediated hypertension (much less characteristic of vasculitides involving smaller vessels).

  • Renal function may be normal unless renal failure supervenes which is often late in the course of classic PAN.

Also microscopic haematuria though uncommon with renal involvement in PAN may well be seen from haemorrhage due to microaneurysms. Harrison 16111 /2007

(Remember that pathology in the kidney in classic PAN is that of Arteritis without Glomerulonephritis). PAN is the answer of choice as if satisfies most diagnostic hint provided in the question.


Q. 16

Neuropathy is not seen in:

 A

Tuberculosis

 B

SLE

 C

Diabetes Mellitus

 D

Polyarteritis nodosa

Q. 16

Neuropathy is not seen in:

 A

Tuberculosis

 B

SLE

 C

Diabetes Mellitus

 D

Polyarteritis nodosa

Ans. A

Explanation:

Answer is A (Tuberculosis):

Tuberculosis is not mentioned as a cause of peripheral neuropathy

Diabetic Mellitus, SLE, PAN and sarcoidosis may all cause peripheral neuropathies.

SLE can cause a distal symmetric sensory or sensorimotor neuropathy and rarely monouritis multiplex.

— Current Neurology Pt/.318 Sarcoidosis may cause a diffuse sensorimotor neuropathy – Current Neurology 1S`/318

PAN may be associated with mononeuropathy multiplex – Current Neurology 1×1/318


Q. 17

Hepatitis B associated with ‑

 A

Wegener’s granulomatosis

 B

Systemic lupus erythmatosus

 C

Polyarteritis nodosa

 D

Sjogren syndrome

Q. 17

Hepatitis B associated with ‑

 A

Wegener’s granulomatosis

 B

Systemic lupus erythmatosus

 C

Polyarteritis nodosa

 D

Sjogren syndrome

Ans. C

Explanation:

Ans. is ‘c’ i.e., Polyatrteritis nodosa

The hepatitis B surface antigen is present in 25% of patients with Polyarteritis Nodosa.

Hepatitis C antibody is present in patients with essential mixed cryoglobinuria and rarely in patients with poly arteritis nodosa.


Q. 18

Fibrinoid necorsis with neutrophilic infiltration is seen in ‑

 A

PAN

 B

Giant cell arteritis

 C

Takayasu arteritis

 D

Wegener’s granulomatosis

Q. 18

Fibrinoid necorsis with neutrophilic infiltration is seen in ‑

 A

PAN

 B

Giant cell arteritis

 C

Takayasu arteritis

 D

Wegener’s granulomatosis

Ans. A

Explanation:

Ans. is ‘a’ i.e., PAN



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