Sjogren syndrome
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Sjogren syndrome
Pathology
Pediatric musculoskeletal conditions
Radial head subluxation (Nursemaid elbow)
Developmental dysplasia of the hip
Legg-Calve-Perthes disease
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Transient synovitis
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Sjogren syndrome
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Musculoskeletal system pathology review
Back pain: Pathology review
Rheumatoid arthritis and osteoarthritis: Pathology review
Seronegative and septic arthritis: Pathology review
Gout and pseudogout: Pathology review
Systemic lupus erythematosus (SLE): Pathology review
Scleroderma: Pathology review
Sjogren syndrome: Pathology review
Bone disorders: Pathology review
Bone tumors: Pathology review
Myalgias and myositis: Pathology review
Neuromuscular junction disorders: Pathology review
Muscular dystrophies and mitochondrial myopathies: Pathology review
Pediatric musculoskeletal disorders: Pathology review
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Sjogren syndrome
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Sjogren syndrome
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| Laboratory value | Result |
| Hemoglobin | 10.2 g/dL |
| Hematocrit | 31% |
| Platelet count | 350,000/mm3 |
| Leukocyte count | 3,000/mm3 |
| Erythrocyte sedimentation rate | 52 mm/h |
| Antinuclear antibodies | Positive |
| Rheumatoid factor | Negative |
| Anti-dsDNA | Negative |
| Anti-Ro/La | Positive |
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Transcript
Contributors
Jahnavi Narayanan, MBBS
Evan Debevec-McKenney
Tanner Marshall, MS
Pauline Rowsome, BSc (Hons)
Sjogren's syndrome, named after Dr. Henrik Sjogren who first identified it, is a common autoimmune disorder, typically occurring in women.
In Sjogren’s syndrome, the body’s immune cells go rogue and start attacking various exocrine glands, which are glands that pour their secretions into a duct; most commonly the salivary glands and the lacrimal, or tear, glands.
Normally, the cells of the immune system are ready to spot and destroy anything foreign pathogens that could cause the body harm.
Immune cells called antigen-presenting cells, which include macrophages and dendritic cells, latch onto pathogens and engulf them - literally swallowing them up.
Pieces of the pathogen called antigens are then presented on a major histocompatibility complex class II molecule, or MHC-class II molecule - which is like a serving platter for antigens. An antigen presenting cell then searches for a T cell that can bind to the antigen.
Once found, binding to the antigen helps activate this T cell, which then releases proinflammatory cytokines, or signaling molecules, that recruit more immune cells - ultimately leading to inflammation.
The exact cause for Sjogren's syndrome is unknown, but it seems to be related to both genetic and environmental factors.
Genetic factors include genes which code for specific types of MHC class II molecules, called human leukocyte antigen, or HLA genes. Specifically HLA- DRW52, HLA- DQA1, or HLA- DQB1.
Environmental factors include an infection of exocrine glands like the salivary and lacrimal glands. Infections can damage the cells of the salivary gland, and expose their cell components, including their DNA, RNA, and histones to circulating immune cells.
Summary
Sjogren's syndrome is an autoimmune disease in which the body's immune cells attack various moisture-producing exocrine glands, which normally pour their secretions into various ducts in the body, such as the salivary and the lacrimal glands. This results in the development of a dry mouth and dry eyes. Other symptoms can include dry skin, a chronic cough, vaginal dryness, numbness in the arms and legs, feeling tired, and muscle and joint pains. Treatment of Sjogren's syndrome typically involves medications to suppress the immune response, like corticosteroids; and also medications that increase the exocrine secretions like pilocarpine.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
- "Update in Sjögren syndrome" Current Opinion in Rheumatology (2000)
- "Novel autoantibodies in Sjögren's syndrome: A comprehensive review" Autoimmunity Reviews (2019)
- "Sjögren's syndrome" The Lancet (2005)
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