04-16 L2 CT disorders

Question 1

Progressive systemic sclerosis (diffuse scleroderma)

s/s

diagnosis

Answer 1

Progressive systemic sclerosis (diffuse scleroderm)

• def.
  
  • on the neck and trunk in additon to face proximal and distal extermities
• ​​​s/s
  
  • raynauds phenomenon followed w/in one year, by edematous or tight skin changes, truncal and acral skin involvmeent, tendon friction rubs
  • early sig incidence of renal interstitial lung, diffuse GI and myocardial disease
• diagnosis
  
  • Anti scl70 antibody (30-50% specific but not sensitive)

Question 2

limited cutaneous sclerosik

s/s

diagnosis

Answer 2

limited cutaneous sclerosis

• def
  
  • _on limbes restircted to the sites distal t_o the elbow and knee but also involving the face &neck
• s/s
  
  • raynaouds phenomenon for yrs, skin involvement limited to hands, face, feet and forearms
  • signifcatn (10-15%) late incidence of PH, w/ or w/o skin calcificatino, GI disease, tenlagniectesse, (CREST sydnrome)
  • renal disease rarely occurs
• diagnosis
  
  • ACA (anticentromere antibody) in 50-60% some series up to 80-90%)
    *

Question 3

CREST syndrome

Answer 3

CREST syndrome

• def
  
  • C=subcutaneous calcinosis
  • R=raynaud’s phenomenon
  • E=esophageal dysmotility
  • S=sclerodactily
  • T=teleangiectases
• diagnosis
  
  • ACA

Question 4

MCTD

def

clinical manifestations

diagnosis

Answer 4

Mixed connective tissue disorder

• Def
  
  • diffuse/generalized CTD
  • clincial features seen in SLE, scleroderma, PM
• Clinical manifestations
  
  • Raynauds w/swollen hands
  • ab_sence of severe renal and CNS disease_
  • more s_evere arthritis_
  • insidious onset PH
• Diagnosis
  
  • anti-U1RNP antibodies
  • Anti RNP

Question 5

Sjorgen’s syndrome

Answer 5

Sjorgen’s syndrome

• def
  
  • systemic autoimmune disease characterized by dry mouth, dry eyes
  • may be primary, or secondary disease accompanies another autoimmune disease most often rheymatoid arthritis or SLE.
• clinical
  
  • parotid enlargement
  • enlarged larimal glands
  • arrthriits
• diagnosis
  
  • positive schirmer test (<5 mm wet in 5 min)
  • normal is 10 mm in 5 min

Question 6

What is a long term concern for patients with sjorgen’s syndrome

Answer 6

• A long term concern for pts with ss is that they develop lymphoma
• SS is characterized by polyclonal-B cell activation as well as by lymphocytic infilration of the exocrine gland
• Most lymphomas arise from a reactive infiltrate called lymphoepithelial sialadenitis or benign lymphoepihelial lesion
• The lifetime risk of non-Hodgken lymphoma (NHL) is approximately 5 %
  
  • the risk is up to 70 higher than general population (different in several series of research)