Connective Tissue Disorders Flashcards
Give examples of connective tissue disorders?
Rheumatoid arthritis
Systemic/ discoid lupus erythematous
Systemic sclerosis
Sjogrens syndrome
What is rheuatmoid arthritis?
Auto-immune condition
Multi-system inflammatory disease of synovial and adjacent tissues
Aetiology of rheumatoid?
Females > males
Peak 35-50 years
What causes rheumatoid?
Auto-immune
Autoantibodies - rheumatoid factor
Issue with looking for rheumatoid factor?
Not very specific
What more accurate marker in rheumatoid?
Anti-CCP
Clinical features of rheumatoid?
Slow onset Pain and stiffness of small joints Fatigue Anaemia Weight loss Muscle loss and wasting Lung problems Lymphadenopathy
Most common joint affects in rheumatoid?
Finger joints
Is TMJ affected in rehumatoid?
in 30% have involvement
What hand signs might see in rheumatoid?
Ulnar deviation/ swan neck deformity
Extra-articular manifestation R.A?
Weight loss Fatigue Lymphadenopathy Rheumatoid nodules Sjogrens
How diagnose rheumatoid?
Bloods - raised ESR/ CRP, anti-CCP positive, rheumatoid factor positive
Look radiographic changes
What guideline for management of rheumatoid?
NICE guidelines
What do NICE suggest management rheumatoid?
Education Exercise - maintain muscle Physio Surgery Dietary advice
When is surgery indicatied for RA?
If progressive deformity
What medications are used RA?
DMARDs
Corticosteroids
Biological agents
Symptomatic relief
What is DMARD and give examples?
Disease modifiying anti-rheumatic drug
e.g methotrexate/ hydroxychlorquine
Give example biological agent?
Anti-TNFa
e.g Infliximab
What medications are given for symptomatic relief of RA?
NSAIDs
What is the oro-facial impact of RA?
Access
Antalo-axial joint dislocation
Impaired manual dexterity - OH
Secondary Sjogrens
What may see is TMJ affected RA?
AOB
Felty’s syndrome can be seen with RA - what might see?
Increased risk oral infections
- oral ulceration/ angular chelitis
What are two forms of lupus erythematous?
Systemic and discoid
What is lupus erythematous?
Immunologically mediated condition
Where does discoid lupus affect?
Skin and oral mucosa
What condition does discoid lupus look similar to?
Lichen planus
How does discoid lupus present on skin?
Scaly, erythaematous patch
Atrophic, hypo-pigmented area
Issue w/ lesions of discoid lupus on skin?
Can be premalignant
How is discoid lupus diagnosed?
Clinical appearance
Biopsy
Circulation auto-antibodies
What auto-antibodies would be present in discoid lupus?
ANA
dsDNA
Management of discoid LE?
Can’t cure
Improve symptoms - difflam/ topical steroids to reduce inflammation
Similar tx lichen planus
What systemic features see in systemic LE?
Malar rash - cheeks/ bridge nose Polyarthirits Photosensitivity Oral lesion Renal/ cardiac lesions
How common is it to have oral lesions w/ systemic LE?
40%
How do oral lesions present in systemic LE?
Uni/bilateral white patch w/ central area erythema/ ulceration
Pharmacological management of systemic LE?
Hydroxychloroquine
Corticosteroids
Cytotoxic - azathioprine/ methoitrexate
What drugs can cause lupus-like reactions?
Carbamazepine Hydralazine Penicillamine Procanimadie Methyldopa
What is systemic sclerosis?
Autoimmune disorder casuing dense collagen deposits in tissues of body
What are types of SS?
Localised
Diffuse
What is localised SS?
Limited skin face, hands and feet
What is diffuse SS?
Extensive skin involvement w/ progression to visceral organs
What has better prognosis diffuse or localised SS?
Localised –10-year survival = 75%
Diffuse – 10 year survival = 55%
What syndrome is linked to localised SS?
CREST syndrome
What See in CREST syndrome?
C - calcinosis (calcium deposits) R - raynauds E - oEsophageal dysfunction S - sclerodactyly T - telangiectasia
What are orofacial manifestations SS?
Facial skin rigidity Thinning lips Loss facial wrinkets Hypo-mobile tongue Dysphagia Xerostomia PDL widening Microstomia
What are issues caused by microstomia in SS?
Poor access OH - increased caries/ perio
Issues: speech, eating
How dx SS?
Difficult - clinical dx
Skin biopsy
PDL widening
Management of SS?
Look improve circulation - nifedipine
Suppress immunity - corticosteroids/ DMARDs
How tx microstomia in SS?
Exercise - stretching
Surgical approach - commissuotomy
What is Sjogrens syndrome?
Autoimmune disease of exocrine glands (salivary/ lacrimal)
Difference between primary and secondary sjogrens?
Primary - absence underlying connective tissue disorder
Secondary - presence of underlying connective tissue disorder (RA/ SLE/ SS)
What see in sjogrens?
Focal lymphocytic infiltration of salivary and lacrimal glands
Is it more common to see sjogrens in SLE or RA?
SLE
SLE 30% pt have secondary sjogrens
RA 15% have secondary sjogrens
Causes of sjogrens?
Genetic predisposition
Viral agent - herpes virus/ retrovirus/ EBV
Pathogenesis of sjogrens?
Lymphocytic infiltration of exocrine glands Hypertrophy of ductal epithelium Formation epimyoepithelial islands Acinar atrophy and fibrosis Hyperactivity of B-cells
Symptoms of xerostomia related sjogrens?
Difficulty swallowing Sensitivity spicy food Altered taste - metalic Burning mucosa Lack taste Salivary gland swelling
Oral sign sjogrens?
Oral mucosa - dry/ atropy/ ulcerated
Tongue - dry/ red/ lobulated
Increased caries
Salivary gland - swelling
What criteria is used for diagnosis of sjogrens?
European diagnostic criteria
How diagnosis sjogrens?
Biopsy of salivary glands
What are the 6 classifications of signs and symptoms in sjogrens?
I - ocular symptoms II - oral symptoms III - ocular signs IV - histopathology V - salivary gland involvement VI - autoantibodies
In regards to classification what makes it primary sjogrens?
Must have 4 out of 6
MUST have positive histopathology and autoantibodies
In regards to classification what makes it secondary sjogrens?
Have underlying CT AND presence of ocular OR oral symptoms AND 2 out of III, IV, V
What are those w/ sjogrens at higher risk of?
Lymphoma - predominantly B cells
Management of Sjogrens?
Palliative -
- Increase lubtrication - salivary substitutes
- Maintain oral/detanl health- OHE/ F- toothpaste
- Review candida status
Therapeutic
- Pilocarpine
- Immunomodulating agents
