Sjogren syndrome: Pathology review
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Sjogren syndrome: Pathology review
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
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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
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Assessments
Sjogren syndrome: Pathology review
USMLE® Step 1 questions
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Questions
USMLE® Step 1 style questions USMLE
of complete
| 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 |
Transcript
Content Reviewers
On your rounds, you see a 55-year-old female named Patricia who presents with fatigue, dry skin, and red eyes. She mentions that she's had a recurrent sensation of sand in her eyes and dry mouth every day for the past three months. She also mentioned that when it’s cold outside, the tips of her fingers turn white and hurt. On examination, there are signs of tooth decay and purpura on both lower extremities. Sialometry was performed, which detected salivary hypofunction.
Ok, so Patricia’s clinical picture is suggestive of Sjogren syndrome. Now, Sjogren syndrome is an autoimmune disorder that mostly affects middle-aged females. The high yield concept here is that the immune system attacks various exocrine glands, especially salivary and lacrimal glands. If Sjogren syndrome is primary or occurs alone, it’s called sicca syndrome. Alternatively, it can be secondary when it is accompanied by other autoimmune diseases like lupus, rheumatoid arthritis, and scleroderma. Now, the exact cause of Sjogren syndrome is unknown, but both genetic and environmental factors are involved.
In Sjogren syndrome, some helper T-cells perceive nuclear components that leak out of dead or damaged cells in the body as antigens. These T-cells become active and proliferate and then activate B-cells which start producing anti-nuclear antibodies, or ANAs, against the nuclear antigens. A high yield fact to remember is that the two types of ANA formed in Sjogren syndrome are anti-SSA/RO and anti-SSB/LA antibodies, which are formed against ribonucleoproteins SS-A and SS-B. Next, both T-cells and antibodies enter the circulation and reach the exocrine glands, where activated T-cells secrete cytokines to recruit even more immune cells. This results in a lymphocytic infiltration of the exocrine glands, which ends up damaging the exocrine gland tissue. Eventually, the secreted cytokines also activate fibroblasts, which produce fibrous tissue that replaces the damaged tissue. The end result is a loss of secretory cells in the glands.
Summary
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Sjögren syndrome" Nature Reviews Disease Primers (2016)
- "Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies" Rheumatology (Oxford) (2016)
- "Immunopathogenesis of Sjögren's Syndrome" Clinical Reviews in Allergy & Immunology (2003)
- "Sjögren's syndrome" Lancet (2005)
- "Clinical, Immunologic, and Molecular Factors Predicting Lymphoma Development in Sjogren’s Syndrome Patients" Clinical Reviews in Allergy & Immunology (2007)
- "Sjögren syndrome" Nature Reviews Disease Primers (2016)
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