Sclerodoma

Question 1

What is scleroderma

Answer 1

o An autoimmune disease that results in wide spread capillary damage and obliterative microangiopathy, leading to fibrosis and impaired function/failure of affected organs
o Early stages are predominantly inflammatory changes while later stages are primarily progressive organ dysfunction.
o More common in young females

Question 2

What are the four types of scleroderma

Answer 2

 Localized: typically limited to skin/muscular changes, mild
 Morphea: thick waxy patches of skin that are scattered and vary in size. Patches disappear spontaneously
 Linear Sclerodoma: Starts as a streak of hard/waxy skin on arm, leg, or forehead. Involves deeper layers and can sometimes involve the joints.
• En coupe de Sabre: A crease that forms on the head or neck
 Systemic Sclerodoma: Affects connective tissues in all parts of the body including internal organs

Question 3

What are the s/s of scleroderma

Answer 3

o Skin: Hard waxy skin patches, may be circular or linear. May develop Raynauds and ulcerations on finger tips and toes
o GI: GERD, malabsorption, diminished peristalsis, obstruction/perf
o GU: Shrinking glomeruli leading to AKI/CKD
o Cardiac: Fibrosis leading to HTN, pericarditis, myocarditis, effusions, CAD
o Pulm: nonspecific interstitial pneumonia, pulm HTN
o Crest Syndrome: Calcinosis + Raynauds + Esophageal dysfunction +Sclerdactyly + Telagiaectasia

Question 4

What is the treatment for scleroderma

Answer 4

o Can only treat symptoms
o Raynauds: CCB
o GERD: Antacids, PPI, H2 blockers
o Kidney disease: ACEi
o Muscle Pain: NSAIDS, prednisone, IVIG, biologics
o Pulm HTN: Prostacyclin drugs (Stenols)

Question 5

What is ankylosing spondylitis

Answer 5

o A autoimmune inflammatory disorder that primarily affects the axial skeleton and joints.
o Joints are eroded overtime at the margins and replaced with fibrocartilage that eventually ossifies.
o Hallmark feature includes involvement of the sacroiliac spine

Question 6

S/s of ankylosing spondylitis

Answer 6

o Pain in the low back and buttocks that is worse in the am and gets better with movement
o Can cause the patient to wake up and have to walk around in the night
o Fatigue

Question 7

Treatment for Ankylosing spondylitis

Answer 7

o NSAIDS- for pain and inflammation
o Sulfasalzine
o Corticosteroids for inflammation
o Biologics: TNF inhibitors (humira, remicade), Cosentex, Stelara