Scleroderma

Question 1

What is scleroderma?

Answer 1

1. Hard skin
   *increased deposition of collagen in interstitium of small arteries and connective tissue
2. Heterogeneous disease
   *systemic connective disorder: will be thickened skin
   *will affect, lung (80%), heart, kidney(60-80%), and GI tract

Question 2

What is the prevalence of scleroderma?

Answer 2

1. 3 to 1 F>M
2. 30-50
3. Black patients have a worse outcome

Question 3

What is the presentation of scleroderma?

Answer 3

1. Raynaud’s phenomenon (initial manifestation)
   *lack of blood supply to the hands and feet when exposed to coldness, can be painful
2. Edema in the fingers and hands
3. Skin thickening
   *will be areas of hyper and hypo pigmentation
4. Visceral manifestations
5. Arthralgias and muscle weakness often
6. Pruritis and edema

Question 4

What are the two primary categories of scleroderma?

Answer 4

1. Diffuse cutaneous
2. Limited cutaneous (CREST)

Question 5

What are the characteristics of diffuse cutaneous scleroderma?

Answer 5

*Worse prognosis
*Skin changes affect trunk and proximal extremities (skin had trouble expanding)
*tendon friction rubs over forearms and shins
*cardiac disease more prevalent
*kidney and lung disease more prevalent

Question 6

What are the characteristics of limited cutaneous scleroderma?(CREST)?

Answer 6

*more benign, less renal
*calcinosis cutis (deposition of insoluble calcium salts in the skin)
*raynaud phenomenon
*espophageal motility disorder
*sclerodatyly (decrease flexion in the fingers, digital ischemia)
*Telangiectasia (spider web looking thingy on skin)

Question 7

What are the head and neck manifestations of scleroderma?

Answer 7

1. Tight skin
2. Thin lips
3. Vertical perioral furrows
   *b/c of the tightening around the mouth, soon the patient wont be able to open their mouth (3 fingers vertical to tell the opening distance)
4. Dysphagia (MC complaint; will be hoarseness)
5. Gingivitis, periodontal thickening

Question 8

What labs should you order if a patient is suspected with scleroderma?

Answer 8

1. CBC/with diff
   *mild anemia is common
2. Serum creatinine (renal dysfunction)
3. Creatine kinase (helps us look at muscles)
4. UA with urine sediment

Question 9

Which labs are usually positive? For dcSSc?

Answer 9

1. Anti-ScL-70 (highly specific)
   *in 1/3 with diffuse, 20% with limited
2. ACA anti-centromere (specific for limited scleroderma 50%) (1% for diffuse)
3. ANA (95%) if negative consider another fibroing illness
4. Anti-RNA polymerase III antibody (highly specific)
5. Abnormal EKG

Question 10

What is the treatment for Scleroderma ?

Answer 10

1. Calcium channel blockers in raynauds
   *nifedipine, losartan
2. PPIs for reflux
3. NSAIDS and steroids for arthralgias and myalgias
4. Hand rehab
5. Esophageal dz meds either liquid or crushed

Question 11

What type of meals should someone eat when they have scleroderma?

Answer 11

1. Small frequent meals if delayed gastric emptying
   *stay upright for 2 hours after
   *laying down will lose the effect of gravity and food can sit

Question 12

What is the prognosis of scleroderma?

Answer 12

1. Organ involvement typically occurs in the 1st 3 years
   *if not then there will be a better prognosis
2. Mortality rate is 4x higher than general population
3. Worse with diffuse, black patients, males, and older patients