Scleroderma
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Scleroderma
Pathology
Bone disorders
Osteomyelitis
Bone tumors
Chondrosarcoma
Osteochondroma
Achondroplasia
Arthrogryposis
Cleidocranial dysplasia
Club foot
Craniosynostosis
Flat feet
Genu valgum
Genu varum
Osteogenesis imperfecta
Pectus excavatum
Pigeon toe
Lordosis, kyphosis, and scoliosis
Osteomalacia and rickets
Osteopetrosis
Osteoporosis
Osteosclerosis
Paget disease of bone
Musculoskeletal injuries and trauma
Degenerative disc disease
Sciatica
Spinal disc herniation
Spondylolisthesis
Spondylolysis
Achilles tendon rupture
Anterior cruciate ligament injury
Iliotibial band syndrome
Meniscus tear
Patellar tendon rupture
Patellofemoral pain syndrome
Sprained ankle
Unhappy triad
Compartment syndrome
Rhabdomyolysis
Carpal tunnel syndrome
Erb-Duchenne palsy
Klumpke paralysis
Sciatica
Thoracic outlet syndrome
Ulnar claw
Winged scapula
Carpal tunnel syndrome
Dislocated shoulder
Erb-Duchenne palsy
Klumpke paralysis
Radial head subluxation (Nursemaid elbow)
Rotator cuff tear
Thoracic outlet syndrome
Ulnar claw
Winged scapula
Musculoskeletal system pathology review
Back pain: Pathology review
Bone disorders: Pathology review
Bone tumors: Pathology review
Gout and pseudogout: Pathology review
Muscular dystrophies and mitochondrial myopathies: Pathology review
Myalgias and myositis: Pathology review
Neuromuscular junction disorders: Pathology review
Pediatric musculoskeletal disorders: Pathology review
Rheumatoid arthritis and osteoarthritis: Pathology review
Scleroderma: Pathology review
Seronegative and septic arthritis: Pathology review
Sjogren syndrome: Pathology review
Systemic lupus erythematosus (SLE): Pathology review
Assessments
Scleroderma
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USMLE® Step 1 questions
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High Yield Notes
13 pages
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Scleroderma
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USMLE® Step 1 style questions USMLE
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Image reproduced from Radiopedia
Which of the following findings is most likely to be seen in this patient?
External References
First Aid
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2016
Anti-centromere antibodies p. 718
scleroderma p. 485
CREST syndrome (limited scleroderma)
Diffuse scleroderma p. 485
Limited scleroderma p. 485
Limited scleroderma (CREST syndrome)
Scleroderma p. 485
labs/findings p. 718, 722
Scleroderma (diffuse)
autoantibody p. 113
Transcript
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Scleroderma refers to systemic sclerosis, a rare autoimmune disorder in which normal tissue is replaced with thick, dense connective tissue. It affects the skin, blood vessels and internal organs. Now, there are two main types of scleroderma: limited cutaneous systemic scleroderma, better known as CREST syndrome; and diffuse cutaneous systemic scleroderma. The word scleroderma comes from the Greek words ‘sclero,’ meaning hard, and ‘derma,’ meaning skin, which points to the hard skin as its most visible feature.
So, normally, when there’s an infection in the body, macrophages will eat some of the invading organisms and break them down. In addition to destroying the pathogen, they also present a fragment of the pathogen, called an antigen, to naive T cells. When the naive T-cells bind to this presented antigen, they mature into T-helper cells, also called CD4+ T-cells, and they go on to help and recruit more immune cells. The T-helper cells release cytokines, which increase the activity of macrophages and attract nearby neutrophils. Macrophages also release cytokines, like TGF-β, that tell fibroblasts to repair damaged tissue after the infection by laying down collagen.
Scleroderma affects women three times more often than men. The exact pathology of scleroderma is not completely understood, but generally, it’s thought that some individuals have a genetic predisposition to scleroderma which is triggered by external triggers. Some known triggers include viral infection by cytomegalovirus and parvovirus B19; exposure to silica dust, organic solvents, and vinyl chloride; and drugs and medications like cocaine, bleomycin, and pentazocine.
Summary
Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease characterized by progressive fibrosis of skin and internal organs such as the gastrointestinal tract, lungs, heart, and kidneys. The exact cause is unknown but is associated with autoimmune processes that lead to excessive collagen production in individuals with a genetic predisposition. Scleroderma can be either limited or diffuse, with symptoms varying according to the organs involved. Treatment options involve immunosuppressants and medications to relieve symptoms and slow the progression of the disease.
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