Connective Tissue Diseases Part 2 Flashcards
What are some general symptoms of connective tissue diseases?
Dry eyes and mouth, Sjogren’s (Sicca) syndrome, poor dental health, mouth ulcers, changes in appearance, skin changes/rashes, Raynaud’s, myalgia, arthralgia
What are some symptoms of complications caused by connective tissue diseases?
SOB, chest pain, GORD, dysphagia, fatigue, tiredness, pain
What are the symptoms of Sjogren’s syndrome?
Dry eyes (gritty feeling), dry mouth, vaginal dryness, bilateral parotid gland enlargement, joint pains, fatigue, unexplained increase in dental caries
What antibodies are involved in Sjogren’s syndrome?
Anti-Ro, Anti-Jo
May also have raised IgG and PV/ESR
What are some complications of Sjogren’s syndrome?
Increased risk of lymphoma, less commonly associated with organ complications than other CTDs
What is systemic sclerosis (scleroderma)?
Multi-system autoimmune disease characterised by vasculopathy, auto-immunity and fibrosis
What are the classic symptoms of systemic sclerosis?
Raynaud’s, skin thickening, dysphagia, GORD, telangiectasia, calcinosis, +/- SOB
What are red flag symptoms in systemic sclerosis?
Onset of Raynaud’s in mid-adulthood
Raynaud’s and SOB/telangiectasia
Digital ulcers/ischaemia
Skin tightness/loss of dexterity of hands
How is the skin involved in diffuse cutaneous systemic sclerosis?
Skin involvement on extremities above and below elbows and knees (plus face and trunk)
How is the skin involved in limited cutaneous systemic sclerosis?
Skin involvement on extremities and only below elbows and knees (and face)
What antibodies are involved in systemic sclerosis?
Anticentromere = 20-30% Antitopoisomerase (anti-Scl-70) Anti-PM-Scl Anti-To/Th Anti-RNA polymerase (20%)
What facial changes occur in systemic sclerosis?
Small mouth with puckering, beaked nose, telangiectasia, thickened/tight skin (lack of wrinkles)
What are some GI complications of systemic sclerosis?
Dysphagia, GORD, gastric antral vascular ectasia (watermelon stomach), small intestinal bacterial overgrowth, malabsorption, fluctuating bowel habit, faecal incontinence
What are some cardio and resp complications of systemic sclerosis?
Interstitial lung disease, pulmonary arterial hypertension (probably vasculopathy), myocardial disease
What are some renal complications of systemic sclerosis?
Scleroderma renal crisis, non-progressive renal dysfunction
What may systemic sclerosis lead to?
Sclerodactyly, digital ischaemia, myositis
What is the classic triphasic presentation of Raynaud’s?
Blanching (white), acrocyanosis (purple/blue), reactive hyperaemia (redness)
What are some treatments for Raynaud’s and recurrent digital ulcers (list from Reynaud’s treatments to treatments for ulcers)?
Calcium channel blockers = nifedipine usually first line
PDE-5 inhibitor = sildenafil
Prostacyclin infusion = iloprost
Endothelin receptor antagonist
What are some drugs used for general Raynaud’s treatment?
Fluoxetine, ARBs, nitrates
What is the main symptoms of pulmonary hypertension?
Predominantly SOB on exertion
What are some risk factors for pulmonary hypertension?
Telangiectasia, anticentromere antibody, increased disease duration (e.g systemic sclerosis)
What are some features of pulmonary hypertension?
Must screen for it yearly in at risk patients, progressive complication that can lead to right heart failure
How is pulmonary hypertension treated?
Aimed at slowing progression PDE 5 inhibitor = sildenafil/tadalafil/vardenafil ERA = bosentan/ambrisentan/macitentan Eproprostenol infusions Oxygen
How does pulmonary fibrosis present?
Progressive SOB, usually associated with a cough, bilateral crackles on chest examination, due to hardening of lung tissue (less pliable and more inefficient)
What are the pulmonary function test results for a patient with pulmonary fibrosis?
Restrictive pattern, reduced forced vital capacity, reduced transfer factor, must be done yearly
How is pulmonary fibrosis treated?
Mycophenolate mofetil = causes immunosuppression
Rarely cyclophosphamide = for very aggressive disease
Ritiximab = second line agent
Nintedanib = antifibrotic
Rarely lung transplant
What are some features of systemic sclerosis renal crisis?
Associated with anti-RNA polymerase III antibody, usually early in disease, high dose of steroids puts people at risk
What occurs in systemic sclerosis renal disease?
Uncontrolled hypertension with proteinuria and rapidly worsening renal function = treated with ACE inhibitors (sometimes need dialysis)
How is skin fibrosis treated?
Methotrexate, mycophenolate
