Connective Tissue Disorders Flashcards
Sjogrens
Autoimmune condition characterised by lymphocytic infiltrates in exocrine organs.
Sjogrens Symptoms
Dry mouth, eyes and vagina. Poor dental health. Myalgia and Arthralgia. Raynauds Parotid gland swelling
Sjogrens associated conditions
Peripheral neuropathy and interstitial lung disease
Increased risk of lymphoma
Immunology in Sjogrens
Anti Ro and Anti La
Raised IgG and plasma viscosity.
Diagnosis of Sjogrens
Ocular dryness
+ve anti-Ro and anti-LA antibodies
Lip gland biopsy
What test is used to determine ocular dryness?
Schirmers test
Sjogrens treatment
Largely symptomatic e.g lubricating eye drops etc
Regular dental care
If the patient with Sjogrens has arthralgia what can be used?
Hydroxychloroquine
Systemic sclerosis
Multisystemic autoimmune disease characterised by vasculopathy autoimmunity + fibrosis.
Systemic sclerosis
Cutaneous Presentation
Raynauds Skin becomes thickened and tight leading to Beaking of the nose, Atrophy of the fingertips Telangiectasia Calcinosis Sclerodactyly
Systemic sclerosis
GI Presentation
Dysphagia, malabsorption, fluctuating diarrhoea/constipation, faecal incontinence
Systemic sclerosis
Pulmonary presentation
Pulmonary hypertension
Pulmonary fibrosis
Systemic sclerosis
Renal Presentation
Accelerated hypertension
Proteinuria
Worsening renal function
How can systemic sclerosis be divided?
Limited- Skin involvement in hand feet and face.
- Linked to anti centromere
- Organ involvement is later
Diffuse- skin involvement includes trunk.
- linked to Anti Scl-70 antibody
- early significant organ involvement.
Systemic Sclerosis treatment
No overall treatment instead tailor to specific systemic involvement.
Systemic sclerosis treatment - Raynauds
Primary Raynauds - Calcium Channel Blockers
- ARB’s Nitrates
Recurrent Digital Ulcers - PDE- 5 Inhibitor Sildenafil
- Bosentan endothelia receptor agonist.
Systemic Sclerosis treatment - Pulmonary hypertension
PDE 5 inhibitor Sildenafil
Bosentan
oOxygen
Systemic Sclerosis treatment - Pulmonary Fibrosis
Mycophenolate Mofetil
Cyclophosphamide ( for aggressive fibrosis)
Rituximab
Nintedanib (anti-fibrotic)
Systemic sclerosis renal presentation and treatment
Associated with Anti RNA polymerase III antibody
Uncontrolled hypertension and renal failure.
ACE or ARB +/- dialysis
Systemic sclerosis
GI treatment
PPI for for reflux to prevent GORD
How does anti-phospholid syndrome manifest?
Recurrent venous or arterial thrombosis and/ or foetal loss.
Common cutaneous presentation of anti-phospholipid syndrome?
Livedo reticularis
Mottled skin
Treatment for anti-phospholipid syndrome
For those with episodes of thrombosis- Aspirin
Recurrent pregnancy loss- Low molecular weight heparin
If patients with antiphospholipid syndrome have positive antibodies but no history of episode of thrombosis what happens?
No anti-coagulation is required simply monitor.
In systemic sclerosis what is CREST?
Calcinosis Raynauds Eosinophilic dysmotility Sclerodactyly Telangactasia
Gout causes
Renal under excretion
Excessive purine intake
Pathogenesis of gout
Deposition of urate crystals within joint.
Uric acid is final step in breakdown of purines.
Where is uric acid usually excreted?
2/3 excreted via renal system
1/3 excreted via gastrointestinal system
What foods are rich in purines?
Alcohol, red meat and seafood.
What causes the precipitation of crystals out into the joint?
Dehydration, trauma, surgery and low temperatures.
Which joint is affected first in gout?
First metatarsalphalangeal joint. Base of the big toe.
How does gout present.
Asymmetrical inflamed joints. Movement is painful and doesn’t alleviate symptoms.
What can gout mimic?
Septic arthritis
What is the blood concentration of uric acid required for gout?
> 0.42 mol/l
How long do symptoms in gout persist?
7-10 days usually with no treatment.
What is gold standard diagnostic method for gout.
Aspirate the joint and observe fluid under cross polarised microscopy.
Also stain with gram culture to exclude septic arthritis.
What do urate crystals look like under polarised microscopy?
Needle shaped negative birefringence.
What are gout tophi?
Deposition of uric acid within the soft tissue. Large white painless lumps which can burst through the skin.
Acute treatment of gout
NSAIDs
Colchicine
Steroids
Gout prophylaxis
Allopurinol however 2-4 weeks after acute episode.
Bridge 2-4 weeks with NSAIDs
Why is there a 2-4 week period after acute when allopurinol can’t be used.
As for some reason it triggers a more severe acute attack if used during acute phase.
What causes pseudo gout?
Calcium pyrophosphate deposits
Pathogenesis of pseudo gout?
Deposition of calcium within hyaline cartilage.
Deposits break out into synovial cavity.
Underlying possible causes of pseudo-gout
Hyperparathyroidism
Renal osteodystrophy
Haemochromatosis
Previous cartilage injury
Pseudo-gout diagnoses
Aspirate and remove fluid and examine under polarised microscopy.
What does pseudo gout look like in microscopy?
Rhomboid crystals
Pseudo-gout treatment
Only acute NSAIDs Colchicine
Hydration
In gout if people can’t tolerate allopurinol what can be used?
Febuxostat
