Scleroderma

Question 1

Definition

Answer 1

Systemic sclerosis. autoimmune chronic inflammatory disease in which the normal tissue is replaced by thick dense CT. affects organs, skin and bv

Question 2

Types of scleroderma-morphea

Answer 2

Limited-linear: crest syndrome - limited to skin & fat

Diffuse: cutaneous systemic scleroderma - skin and viscera (lung, heart, kidney, GIT)

Question 3

Pathophysiology

Answer 3

Triggers: viral, medications, silica dust,…
injury to endothelium, adhesion molecules expressed allows T cells to get into tissue and release cytokines which also activates fibrobalsts that produce collagen and causes the stiffness of tissue and fibrosis whcih also reduces blood flow to tissue
Activated B cells produce antibodies: ANA, ANTI-SCL70, ARA, ACA

Question 4

General signs and symptoms

Answer 4

Skin lesions that move from the arm to the shoulders and face
early stage: swollen doughy skin & Fibrous plaques with violet edge
late stage: tight, shiny skin & fibrosis, hyperpigmentation
Sclerodyctyly: ulcerations and claw hands
Microstomia, beaked nose, Reynaud’s phenomena, shortening of tongue bridle
Esophageal dysfunction, GERD, Malabsorption
Pulmonary HTN, fibrosis, hypertrophy of RV, renal HTN
Organ damage occurs early with diffuse form

Question 5

signs & symptoms in Limited form (CREST)

Answer 5

Reynaud’s phenomena
Calcinosis
scelrodactyly
Esophageal dysmotility (hourglass sign)
Telangiectasia
organs damage comes later
early stage: swollen doughy skin & Fibrous plaques with violet edge
late stage: tight, shiny skin & fibrosis, hyperpigmentation
Linear scleroderma (en coup de saber) saber strike
high borellia ab titer

Question 6

Diagnosis

Answer 6

Histology: normal epidermis, thick dermis, thin subcutaneous fat, decreased number of sweat glands, sebaceous glands, hair follicles, skin calcinosis
Antibodies
Organs tests
Borellia antibodies

Question 7

Treatment

Answer 7

Immunosuppressants
Corticosteroids
vasoactive medications (trental, Adalat)
Bath-PUVA
Physical therapy
CCB, PPI, NSAIDS, ACEI, Anticoagulants, monitor BP

Question 8

Case Presentation