Scleroderma

Diffuse cutaneous SSc is associated with 

Late stages of limited systemic sclerosis patients may develop

• Pulmonary arterial hypertension (PAH), 
• Interstitial lung disease, 
• Hypothyroidism, 
• Primary biliary cirrhosis 

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

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                                                       

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.

Answer is C (Anticentromere antibodies are characteristic):

Antitopoisomerase antibodies (Scl 70) are characteristic of diffuse systemic sclerosis and not Anticentromere antibodies.

Subsets of systemic sclerosis (SSC): Limited cutaneous SSC versus diffuse cutaneous:

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