Systemic Sclerosis

Systemic Sclerosis

Q. 1

Diffuse cutaneous systemic sclerosis is characterised by:

 A

Early pulmonary fibrosis

 B

Raynauds phenomenon

 C

Calcinosis cutis

 D

Long term prognosis is better

Q. 1

Diffuse cutaneous systemic sclerosis is characterised by:

 A

Early pulmonary fibrosis

 B

Raynauds phenomenon

 C

Calcinosis cutis

 D

Long term prognosis is better

Ans. A

Explanation:

Diffuse cutaneous SSc is associated with 

1. Progressive skin induration
2. Early pulmonary fibrosis and acute renal involvement. 
Limited cutaneous SSc have 
1. Long-standing Raynaud’s phenomenon before other manifestations of SSc appear. 
2. Skin involvement remains limited to the fingers (sclerodactyly) 
 
A subset of patients with limited systemic sclerosis have 
a) calcinosis cutis, 
b) Raynaud’s phenomenon, 
c) esophageal dysmotility, 
d) sclerodactyly
e) telangiectasia
(Termed CREST syndrome)
Long term prognosis is poor in diffuse variety than limited systemic sclerosis.
Ref: Harrison, E-18, P- 2757.

 


Q. 2

In late stages of systemic sclerosis may have all the given features, EXCEPT:

 A

Pulmonary artery hypertension

 B

Interstitial lung diserase

 C

Primary biliary cirrhosis

 D

Primary sclerosing cholangitis

Q. 2

In late stages of systemic sclerosis may have all the given features, EXCEPT:

 A

Pulmonary artery hypertension

 B

Interstitial lung diserase

 C

Primary biliary cirrhosis

 D

Primary sclerosing cholangitis

Ans. D

Explanation:

Late stages of limited systemic sclerosis patients may develop

  • Pulmonary arterial hypertension (PAH), 
  • Interstitial lung disease, 
  • Hypothyroidism, 
  • Primary biliary cirrhosis 
 
Raynaud’s phenomenon and other typical features of systemic sclerosis occur in the absence of detectable skin thickening in some patients. This syndrome is been termed SSc sine scleroderma.
Ref: Harrison, E-18, P- 2757.

Q. 3

Limited cutaneous systemic sclerosis have the following characteristic autoantibody positivity:

 A

Anti Topoisomerase I

 B

Anti-RNA polymerase III

 C

Scl-70

 D

Anticentromere

Q. 3

Limited cutaneous systemic sclerosis have the following characteristic autoantibody positivity:

 A

Anti Topoisomerase I

 B

Anti-RNA polymerase III

 C

Scl-70

 D

Anticentromere

Ans. D

Explanation:

Subset of systemic sclerosis limited cutaneous scleroderma has the following features:

  • Skin involvement-Indolent onset and progression. Limited to fingers.
  • Raynaud’s phenomenon-Precedes skin involvement; associated with critical ischemia
  • Pulmonary artery hypertension-Frequent, late, may be isolated
  • Pulmonary fibrosis – rare
  • scleroderma renal crisis-Very rare
  • Calcinosis cutis-Frequent, prominent
  • Characteristic autoantibodies-Anti Centromere
In Diffuse systemic sclerosis Characteristic autoantibodies-Anti Topoisomerase I (Scl-70), anti-RNA polymerase III
Ref: Harrison, E-18, P- 2758.

Q. 4

All of the following statements regarding systemic sclerosis are TRUE, EXCEPT:

 A

Increased serum antibodies to human cytomegalovirus are seen

 B

Eosinophilia myalgia syndrome is a subtype of systemic sclerosis

 C

The incidence of SSc is increased among miners exposed to silica

 D

Bleomycin, pentazocine and cocaine may produce systemic sclerosis like illness

Q. 4

All of the following statements regarding systemic sclerosis are TRUE, EXCEPT:

 A

Increased serum antibodies to human cytomegalovirus are seen

 B

Eosinophilia myalgia syndrome is a subtype of systemic sclerosis

 C

The incidence of SSc is increased among miners exposed to silica

 D

Bleomycin, pentazocine and cocaine may produce systemic sclerosis like illness

Ans. B

Explanation:

Patients with systemic sclerosis:

  • Have  increased serum antibodies to human cytomegalovirus (hCMV) 
  • Evidence of human parvovirus B19 infection is seen 
  • It is more  in  miners exposed to silica.
  • It is more in polyvinyl chloride, epoxy resins, and aromatic hydrocarbons including toluene and trichloroethylene exposures. 
  • Bleomycin, pentazocine and cocaine are implicated with systemic sclerosis                                                          
  • Women with silicone breast implants-more risk                                                       
Eosinophilia-myalgia syndrome (EMS) have marked eosinophilia, severe myalgia and scleroderma-like chronic skin lesions. It is linked to the consumption of L-tryptophan used as dietary supplements.
It is not a subtype of systemic sclerosis. Similar features also seen with toxic oil syndrome.
Ref: Harrison,E-18,P- 2758.

Q. 5

Pulmonary hypertension may occur in all of the following conditions, EXCEPT:

 A

Toxic oil syndrome

 B

Progressive systemic sclerosis

 C

Sickle cell anaemia

 D

Argemone mexicana poisoning

Q. 5

Pulmonary hypertension may occur in all of the following conditions, EXCEPT:

 A

Toxic oil syndrome

 B

Progressive systemic sclerosis

 C

Sickle cell anaemia

 D

Argemone mexicana poisoning

Ans. D

Explanation:

Argemone mexicana poisoning results from use of edible oils adulterated with argemone mexicana seed oil the clinical condition is known as epidemic dropsy.

Sanguinarine and dihydrosanguinarine are two major toxic alkaloids of argemone oil, which cause widespread capillary dilatation, proliferation and increased capillary permeability.

When mustard oil is adulterated deliberately (as in most cases) or accidentally with argemone oil, proteinuria (specifically loss of albumin) occurs, with a resultant edema.

Pulmonary hypertension is not described in argemone mexicana poisoning. All other conditions are well known to produce pulmonary hypertension.


Q. 6

Raynaud’s phenomenon is seen in –

 A

SLE

 B

Systemic sclerosis

 C

DM

 D

a and b

Q. 6

Raynaud’s phenomenon is seen in –

 A

SLE

 B

Systemic sclerosis

 C

DM

 D

a and b

Ans. D

Explanation:

Ans. is ‘a’ i.e., SLE; ‘b’ i.e., Systemic sclerosis

Raynaud’s phenomenon

  • Raynaud’s phenomenon refers to episodic pallor of digits of the hands or feet. There is a typical sequence in change in colour from white, through blue, to red.
  • Skin discoloration occurs because an abnormal spasm of the blood vessels causes a diminished blood supply to the local tissues.
  • Initially, the digits involved turn white because of the diminished blood supply. The digits then turn blue because of prolonged lack of oxygen cyanosis develop. Finally, the blood vessels reopen, causing a local “flushing” phenomenon, which turns the digits red.

o Most cases of Raynaud’s phenomenon are primary, due to exaggerated vasomotor responses. There is no structural abnormality in the arterial wall, except later in course when thickening of intima is seen.

  • Secondary Raynaud’s phenomenon is associated with SLE, systemic sclerosis, Atherosclerosis, Buerger’s disease, RA.

Q. 7

Features of systemic sclerosis are –

 A

Calcinosis

 B

Sclerodactyly

 C

Raynaud’s phenomena

 D

All

Q. 7

Features of systemic sclerosis are –

 A

Calcinosis

 B

Sclerodactyly

 C

Raynaud’s phenomena

 D

All

Ans. D

Explanation:

Ans. is ‘a’ i.e., Calcinosis; ‘b’ i.e., Sclerodactyly & ‘c’ i.e., Raynaud’s phenomena

Systemic sclerosis (scleroderma)

  • Systemic sclerosis is a chronic disease of unknown etiology characterized by abnormal accumulation of fibrous tissue in skin and multiple organs.
  • Systemic sclerosis is primarily a disease of female.

o The most common age is 30-50 years.                                                                  

(Note – Robbin’s has given 50-60 years. I went through many other books and various articles. According to them the age group is 30-50 years).

o It is characterized by excessive fibrosis throughout the body.

o The skin is most commonly affected, but the GIT, kidney, heart, muscles and lungs also are involved. Classification

  • Systemic sclerosis (scleroderma) has been classified into two major categories –

1.Diffuse scleroderma

o Wide spread skin involvement at onset.

  • Rapid progression and early skin involvement.

2. Limited scleroderma

  • Skin involvement is confined to fingers, forearm and face.

o Visceral involvement is late.

  • Progression is slow and relatively benign.
  • Some patients with limited disease also develop a combination of calcinosis, sclerodactyly, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly and telengectasis, called the CREST syndrome. Note ‑

o Calcinosis                                        –> Subcutaneous calcification of finger and along extensor surface of forearm.

o Raynaud phenomenon                       —> Episodic vasoconstriction of arteries and arterioles of the extremities.

o Sclerodactyly                                    –> Skin fibrosis and thickening limited to fingers and toes.

o Telengectasis                                  —> Dilated capillaries.


Q. 8

ANA (antinuclear antibody) is seen in all except‑

 A

ST E

 B

RA

 C

Sjogren’s syndrome

 D

Systemic sclerosis

Q. 8

ANA (antinuclear antibody) is seen in all except‑

 A

ST E

 B

RA

 C

Sjogren’s syndrome

 D

Systemic sclerosis

Ans. B

Explanation:

Ans. is ‘b’ i.e., RA


Q. 9

Antitopoismerase I is mark of-

 A

Systemic sclerosis

 B

Classic Polyarteritis nodosa

 C

Nephrotic syndrome

 D

Rheumatoid arthritis

Q. 9

Antitopoismerase I is mark of-

 A

Systemic sclerosis

 B

Classic Polyarteritis nodosa

 C

Nephrotic syndrome

 D

Rheumatoid arthritis

Ans. A

Explanation:

Ans. is ‘a’ i.e., Systemic sclerosis


Q. 10

Antinuclear antibodies are seen in –

 A

SLE

 B

Systemic sclerosis

 C

Morphea

 D

All

Q. 10

Antinuclear antibodies are seen in –

 A

SLE

 B

Systemic sclerosis

 C

Morphea

 D

All

Ans. D

Explanation:

Ans. is ‘a’ i.e., SLE; ‘b’ i.e., Systemic sclerosis & ‘c’ i.e., Morphea


Q. 11

A 35 year old lady complains dysphagia, Raynaud’s phenomenon, sclerodactyly. Investigations show antinuclear antibody. The likely diagnosis is :

 A

Systemic lupus erythematosis

 B

Systemic sclerosis

 C

Mixed connective tissue disorder

 D

Rheumatoid arthritis

Q. 11

A 35 year old lady complains dysphagia, Raynaud’s phenomenon, sclerodactyly. Investigations show antinuclear antibody. The likely diagnosis is :

 A

Systemic lupus erythematosis

 B

Systemic sclerosis

 C

Mixed connective tissue disorder

 D

Rheumatoid arthritis

Ans. B

Explanation:

Answer is B (Systemic Sclerosis) :

Features of ‘CREST’ syndrome in association with antinuclear antibodies is sufficient to establish diagnosis of limited form of ‘systemic sclerosis’

CREST Syndrome (Limited systemic sclerosis)

C          Calcinosis

R          Raynaud’s syndrome

E           Esophageal dysmotility

S           Sclerodactyly

T          Telangiectasia

Antinuclear antibodies of ‘Anticentromere’ type are characteristic.

Remember:

Manifestations

Diffuse

Limited (CREST)

Skin involvement

Distal and proximal extremities, face, trunk

Distal to elbow, face

Raynaud’s phenomenon

Onset within 1 year or at time of skin

changes

May precede skin disease by years

Organ involvement

Pulmonary (interstitial fibrosis);

Renal (renovascular hypertensive crisis)

Gastrointestinal

Cardiac

Gastrointestinal

Pulmonary arterial hypertension after 10-15 years of

disease in <10% of patients;

Biliary cirrhosis

Antinuclear antibodies

Anti-topoisomerase 1

Anticentromere


Q. 12

Features of systemic sclerosis include all of the following, Except:

 A

Calcinosis

 B

Sclerodactyly

 C

Hyperpigmentation (Melanin deposition)

 D

More common in young patients

Q. 12

Features of systemic sclerosis include all of the following, Except:

 A

Calcinosis

 B

Sclerodactyly

 C

Hyperpigmentation (Melanin deposition)

 D

More common in young patients

Ans. D

Explanation:

Answer is D (More common in young patients):

Systemic sclerosis is primarily a disease of adults and the mean age of presentation is around 50 years.

Systemic sclerosis is not a disease of young individuals

The mean age of presentation of systemic sclerosis is 50 years — Current Diagnosis & Treatment in Rheumatology 2nd/228 The peak incidence of systemic sclerosis is 50-60 years — Robbins 7th/239

The most common age of onset in the range of 30-50 years — Harrison 171h/2097

Systemic sclerosis is more common in women

Like other connective tissue disorders systemic sclerosis shows a female predominance- Harrison 17th/2097 Systemic sclerosis is primarily a disease of women (female to male ratio is 3: I) — Robbin,s

Systemic sclerosis may present with Calcinosis, Sclerodactyly and hyperpiamentation

The skin of extremities and trunk may be darkly pigmented. Diffuse tanning in the absence of sun exposure may be a very early manifestation of skin involvement — Harrisons

Calcinosis and sclerodactyly are part of the ‘Crest syndrome’ in limited systemic sclerosis

 

CREST syndrome

C

Calcinosis

R

Raynauld’s

E

Esophageal dysmotility

S

Sclerodactyly

T

Telengactasia



Q. 13

Multiple connective tissue disorder not associated with ‑

 A

SLE

 B

Systemic sclerosis

 C

Polymyositis

 D

Sjogren’s syndrome

Q. 13

Multiple connective tissue disorder not associated with ‑

 A

SLE

 B

Systemic sclerosis

 C

Polymyositis

 D

Sjogren’s syndrome

Ans. D

Explanation:

Ans. is ‘d’ i.e., Sjogren’s syndrome

Mixed connective tissue disorder :‑

  • The term mixed connective tissue disease is used to describe a disease with clinical features that are a mixture of the features of SLE, systemic sclerosis, and Polymyositis.
  • The disease is characterized serologically by high titers of antibodies to ribonucleoprotein particle-containing Ul ribonucleoprotein.
  • Typically, mixed connective tissue disease presents with synovitis of the fingers, Raynaud phenomenon and mild myositis, but renal involvement is modest and there is a good response to corticosteroids, at least in the short term.
  • Serious complications of mixed connective tissue disease include pulmonary hypertension, interstitial lung disease, and renal disease.

Q. 14

With these features of nail , it may be seen in a patient with?

 A

systemic sclerosis

 B

viral infection

 C

HIV

 D

None of the above

Q. 14

With these features of nail , it may be seen in a patient with?

 A

systemic sclerosis

 B

viral infection

 C

HIV

 D

None of the above

Ans. A

Explanation:

Longitudinal ridging, ragged cuticle and periungual telangiectasia in a patient with systemic sclerosis.


Q. 15

ANA (antinuclear antibody) is seen in all except‑

 A

SLE

 B

RA

 C

Sjogren’s syndrome 

 D

Systemic sclerosis

Q. 15

ANA (antinuclear antibody) is seen in all except‑

 A

SLE

 B

RA

 C

Sjogren’s syndrome 

 D

Systemic sclerosis

Ans. B

Explanation:

Ans. is ‘b’ i.e., RA 



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