All are true about systemic sclerosis except:
Ans: A (Genetic component) Ref: Harrison's 18th edn, pg 2757Explanation:ANA is positive in allScl-70 and anticentromere abs specific for sclerodermaScl-70 mostly seen in diffuse cutaneous sclerodermaAnticentromere abs seen more in limited cutaneous scleroderma and Pulm artery hypertensionAbs to b2GPI - Ischemia of fingers1.6% prevalent of family historyHigh risk of other autoimmune disorders like SIX, RASystemic Sclerosis(Aka Systemic Scleroderma)DiffuseMost common type in childhoodSymmetric thickening and hardening of the skin (sclerosis) with fibrous and degenerative changes of visceraLimitedRare in childhoodPreviously known as CREST (calcinosis cutis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) syndromeSystemic SclerosisThe skin manifestations of SSc include.Early phase of edema.Induration and fibrosis of skinLoss of subcutaneous fat, sweat glands and hair follicles.Atrophic skin becomes shiny and waxy in appearance.Flexion contractures develop at the elbows, hips and knees associated with secondary muscle weakness and atrophy.Small oral stoma with decreased mouth aperture.Skin ulceration over pressure points, such as the elbows, may be associated with subcutaneous calcifications.Severe Ray naud phenomenon causes ulceration of the fingertips with subsequent loss of tissue pulp and tapered fingers (sclerodactyly).Resorption of the distal tufts of the distal phalanges may occur (acro-osteolysis).Hyperpigmented postinflammatory changes surrounded by atrophic depigmentation gives a salt-and-pepper appearance.Pulmonary disease is the most common visceral manifestation of SSc anti includes both arterial and interstitial involvement (alveolitis).Symptoms ranee from asymptomatic disease to exercise intolerance, dyspnea at rest, and rightsided heart failure.Pulmonary arterial hypertension (PAH) is a poor prognostic sign.Gastrointestinal tract disease is seen in 25% of children with the disease.Common manifestations include esophageal and intestinal dysmotility resulting in dysphagia, reflux, dyspepsia, gastroparesis, bacterial overgrowth, dilated bowel loops and pseudoobstruction. dental caries, as well1 as malabsorption and failure to thrive.Renal arterial dise associated with arrhythmias, ventricular hypertrophy and decreased cardiac function,Mortality from ase can cause chronic or severe episodic hypertension.Cardiac fibrosis is juvenile systemic sclerosis is most commonly a result of cardiopulmonary disease.
Genetic component
Calcinosis cutis
Pulmonary artery hypertension
Anticentromere antibodies
Disorder of Metabolism & Connective Tissue