Connective Tissue Disease Flashcards
Explain the fundamental characteristics of connective tissue diseases and why they’re considered a “heterogeneous family” of conditions.
Connective tissue diseases are characterized by:
- Varied origins (both inherited and acquired forms)
- Autoimmune basis with specific antibodies targeting connective tissue components
- Multi-system involvement affecting: skin, muscle, joints, tendons, ligaments, bone, cartilage, eyes, blood vessels
- Female predilection
- Management typically involves immunosuppression
Rheumatology-led care with multidisciplinary involvement
The heterogeneity comes from the diverse presentation patterns, varying affected tissues, and different underlying mechanisms, despite sharing some common features.
Compare and contrast Primary and Secondary Sjögren’s syndrome, including their diagnostic criteria according to ACR/EULAR 2016.
Primary Sjögren’s:
* Occurs independently
* Primarily presents with sicca symptoms
* Diagnostic criteria must be met independently
Secondary Sjögren’s:
* Occurs alongside another CTD (e.g., RA, SLE)
* May have additional symptoms related to primary condition
Diagnostic Criteria (ACR/EULAR 2016):
1. Oral: Unstimulated salivary flow ≤0.1ml/min
2. Ocular: Schirmer’s test ≤5mm/5 mins OR
Ocular staining score ≥5
3. Serology: Positive anti-Ro or anti-La antibodies
4. Histopathology: Labial salivary gland biopsy with lymphocytic focus score ≥1
Describe the comprehensive oral manifestations of Sjögren’s disease and explain their underlying mechanisms.
Direct effects of reduced saliva:
Oral discomfort
Dysarthria (speech difficulty)
Dysphagia (swallowing difficulty)
Poor denture retention
Dysgeusia (taste alterations)
Secondary complications:
Increased caries risk (reduced buffering/cleansing)
Candidal infections (altered oral environment)
Salivary gland swellings
Sialadenitis (acute salivary gland infection)
Long-term complications:
Risk of salivary gland lymphoma (non-Hodgkin’s)
Requires regular ultrasound surveillance
How is sjogren’s disease managed?
Conservative:
* Mouth: frequent sips of water, saliva substitutes, dental prevention
* Eyes: eye drops, warm compresses
Medical:
* Hydroxychloroquine - immunosuppressant, side effects: retinopathy, oral pigmentation
* DMARDs - specialist led
* Pilocarpine stimulates salivary and lacrimal secretions, side effects +++
Follow-up
* Ultrasound surveillance for lymphoma
Explain the pathophysiology of SLE and how it manifests in different body systems.
Pathophysiology:
- Autoimmune damage targets proteins in cell nucleus
- Generates chronic inflammatory response via complement cascade
- 90% female predominance
Multi-system manifestations:
- Cutaneous: “Butterfly” rash on face, Hair loss
- Musculoskeletal: Joint/muscle pain
- Oral: Ulceration
- Vascular: Raynaud’s phenomenon
- Renal: Impaired kidney function
- Hematologic: Anemia
What auto-antibodies are active in SLE?
- ANA (antinuclear antibody)
- Anti-DNA
How is SLE managed?
- Hydroxychloroquine
- DMARDs (methotrexate, mycophenolate, cyclophosphamide)
- Biologics -Rituximab (CD20, B cells)
Detail the dental implications of SLE and explain how each aspect affects dental management.
Secondary Sjögren’s syndrome:
Medication-related:
* Hydroxychloroquine: Monitor for oral pigmentation
* Methotrexate: Risk of oral ulceration and bone marrow suppression
* General immunosuppression: Increased infection risk
Systemic complications:
* Renal impairment affects drug metabolism
* Need to check BNF for medication adjustments
* May require antibiotic prophylaxis
Direct manifestations:
- Oral ulceration requires symptomatic management
- Increased risk of periodontal disease
Explain the CREST syndrome components in systemic sclerosis and their clinical significance.
CREST syndrome components:
Calcinosis:
Calcium deposits under skin, especially fingertips
Affects manual dexterity
Raynaud’s phenomenon:
Color changes in fingers/toes with cold exposure
Represents vascular dysfunction
Esophageal dysmotility:
Fibrosis causing swallowing difficulties
Results in acid reflux
Sclerodactyly:
Skin thickening around fingers
Can lead to ulceration
Name derives from “skleros” (hard) + “daktylos” (digit)
Telangiectasia:
Dilated blood vessels in skin
Results from inflammation
can also result in microstomia (small mouth due to fibrosis)
What is systemic sclerosis?
What autoantibodies are present?
Autoimmune inflammation and fibrosis of skin, mucosa and organs
* ANA antibodies (non-specific)
* Anti-centromere / anti-Scl-70 antibodies
How is systemic sclerosis managed?
non-pharmacological, pharmacological
Non-pharmacological:
* skin stretching, physiotherapy, occupational therapy, avoiding cold triggers (Raynaud’s)
Pharmacological:
* DMARDs
* Biologics
Analyze the comprehensive dental implications of systemic sclerosis and develop appropriate management strategies.
Access issues:
* Microstomia (small mouth) from facial tissue fibrosis
* Requires adaptation of dental techniques
* May need modified instruments/approaches
Oral hygiene challenges:
* Calcinosis and sclerodactyly affect manual dexterity
* Need for adapted oral hygiene aids
* More frequent professional cleanings
Dental complications:
* Acid reflux from esophageal dysmotility leads to dental erosion
* Requires preventive strategies
* May need more frequent monitoring
Secondary conditions:
- Secondary Sjögren’s syndrome
-Need comprehensive dry mouth management
Medication considerations:
- Monitor for side effects of DMARDs and biologics
- May require antibiotic prophylaxis
Explain the pathophysiology of GCA and why it’s considered a dental emergency.
- Granulomatous inflammation in artery wall
- Affects temporal artery and other external carotid branches
- Blocks downstream blood flow
- Can affect posterior ciliary arteries leading to optic nerve ischemia
- Often associated with polymyalgia rheumatica (~50% of cases)
Emergency aspects:
* Risk of permanent blindness if untreated
* Requires immediate high-dose prednisolone (40mg)
* Treatment shouldn’t wait for diagnostic confirmation
* Affects those 50+ years old
* Can present as dental pain or TMD
Detail the diagnostic approach for GCA and explain the significance of each finding.
clinical pres, lab, imaging, histopathology
Clinical presentation:
* Headache
* Scalp tenderness
* Jaw claudication
* Visible temporal artery swelling
* Visual symptoms (blurred/double vision)
* Systemic symptoms (fatigue/fever/weight loss)
Laboratory findings:
* Elevated inflammatory markers (CRP, ESR)
Imaging:
* Ultrasound showing “halo sign”
Histopathology:
Temporal artery biopsy showing:
* Granulomatous inflammation
- Multinucleated giant cells
- Must be >1cm due to “skip lesions”
Compare and contrast the pathophysiological mechanisms proposed for fibromyalgia and explain their clinical implications.
Central Sensitization:
* Altered CNS pain processing
* Lower pain perception threshold
* Explains widespread pain symptoms
Neuroendocrine changes:
* Altered cortisol levels
* Reduced REM sleep
* Contributes to fatigue and cognitive symptoms
Peripheral nerve alterations:
* Modified pain signaling
* Contributes to heightened sensitivity
Immune dysfunction:
* May explain inflammatory symptoms
Psychiatric overlap:
* Depression comorbidity
* Bidirectional relationship with symptoms
Analyze the relationship between fibromyalgia and various orofacial pain conditions.
what are the management implications
TMD overlap:
Shared central sensitization mechanisms
Similar pain processing alterations
May require modified management approaches
Burning mouth syndrome:
Common pain mechanisms
Need to differentiate from other causes
Other orofacial presentations:
Persistent Idiopathic Facial Pain
Atypical Odontalgia
Oral pain without clear organic cause
Dysgeusia
Subjective xerostomia
Management implications:
Requires multifaceted approach
Focus on pain management strategies
May need psychological support
Often requires interdisciplinary care
