Help identify this distinct subset of scleroderma!! (MCQ)

Question

A 49-year-old man, full-time ceramic sculptor, presented with increasing numbers of firm indurated plaques that produced a band-like tightening on his abdomen, which diminished his appetite and impaired his ability to breathe. His disease progressed to involve his upper thighs and lower legs, limiting his ability to walk.
He also complained of extensive esophageal reflux but no history suggestive of Raynaud's phenomenon.
On physical examination, the upper extremities demonstrated diffuse firm, indurated plaques from the dorsal hands to the shoulders .
There was extensive tightening and induration of the skin on the abdomen, back upper buttock, and the lower extremities from the hips down to the dorsal feet, with sparing of his face.
He was suspected of having a distinct subset of scleroderma characterised by the absence of vasculopathy, a male predisposition and more frequent tower gastrointestinal in votvements .

Which antibody when negative , helps identify this distinct subset of scleroderma?

  • A. ANCA.
  • B. Rheumatoid factor.
  • C. Anti centromere .
  • D. Antinuclear antibody.
  • E. Anti Fibroblast antibodies.

Check your answer ...


More...

Explanation

Systemic sclerosis (SSc) patients who are ANA NEGATIVE constitute a distinct subset of SSc with less vasculopathy, a greater proportion of males and more frequent lower gastrointestinal involvement.

Serologic testing for autoantibodies can be helpful in diagnosing and classifying systemic sclerosis.
Antinuclear antibodies (ANAs) are found in patients whose immune system is predisposed to cause inflammation
against their own body tissues.

Serologic testing for autoantibodies can be helpful in diagnosing and classifying systemic sclerosis.
However. none of the serologic tests is sensitive enough to independently exclude disease.

Antinuclear antibody (ANA) is positive in 60% to 80% of patients with scleroderma.
Anticentromere antibodies (ACAs) are found in roughly 60% to 80% of patients with limited cutaneous systemic sclerosis and only rarely in patients with Diffuse cutaneous systemic sclerosis.
Antibodies to topoisomerase-1 (Scl-70 antibodies) are present in about 30% of patients with dcSSc.

The presence of either ACA or anti-Scl-70 is highly specific (95%-99%) for the diagnosis of lcSSc and dcSSc,
respectively. These antibodies are only rarely present in healthy subjects and in patients with other rheumatologic diseases.

The presence of ACA or anti-Scl-70 antibodies is also highly specific for underlying systemic sclerosis
in patients presenting initially with isolated Raynaud's phenomenon and therefore may be helpful
when Raynaud's phenomenon is the initial manifestation


A Case of Localized Scleroderma in a Sculptor and His Wife
https://insights.ovid.com/pubmed?pmid=19955998



Last Updated on

>