MUSCULOSKELETAL & RHEUMATOLOGY Flashcards
what is Systemic Lupus Erythematosus (SLE)?
chronic autoimmune disease that can affect any part of the body, including the skin, joints, kidneys, brain, and other organs
what is the clinical criteria of SLE?
- malar (butterfly)rash, discoid rash, photosensitivity rash
- oral ulcers, alopecia
- arthritis (symmetrical)
- kidney disorder (proteinuria/cellular casts/high BP),
- neurological disorder (seizures or psychosis),
- hematologic disorder (hemolytic anemia (Coombs positive), lymphopenia, or thrombocytopenia)
- other organ involvement
what are tests done for SLE?
- Anti-dsDNA and Anti-Smith (Anti-Sm) Antibodies - specific
- Antinuclear Antibody (ANA) Test - positive, less specific
- complement factors, C3, C4 - low is LSE
- FBC, CRP, ESR - indicate inflammation and anaemia
- urinanalyis - kindey involvement
- imaging eg xray for other organ involvement
what is the treatment/management of SLE?
- (NSAIDs): For joint pain and stiffness.
- Antimalarial Drugs (Hydroxychloroquine): skin and joint symptoms.
- Corticosteroids and Immunosuppressive Agents: For more severe disease manifestations
- cytotoxic drugs
- Biologics: belimumab
- Lifestyle Modifications: sun protection, exercise, healthy diet.
- stem cell transplant
- Identify patients with phospholipid antibodies
- assess atherosclerosis/major organs
what is Raynaud’s phenomenon?
blood stops flowing properly to your fingers and toes
what causes primary vs secondary Raynaud’s phenomenon?
primary - unknown, absence of another underlying disease
secondary - autoimmune diseases (e.g. SLE, scleroderma, rheumatoid arthritis), injuries, drugs, vascular damage
what kind of vascular damage can cause Raynaud’s phenomenon?
Atherosclerosis
Frost bite
Vibrating tools
what are the 2 subtypes of systemic slerosis/scleroderma?
- Limited cutaneous systemic sclerosis (lcSSc) (AKA CREST)
- Diffuse cutaneous systemic sclerosis (dcSSc)
what are the main features of systemic sclerosis?
Vasculopathy
Excessive collagen deposition
Inflammation
Auto-antibody production
what is the diffrence between limited and diffuse systemic sclerosis?
limited:
- Long history of Raynaud’s phenomenon
- skin thickening at hands, arms, and face
- slow progressing
Diffuse:
- Short History of Raynaud’s phenomenon
- thickening extends to trunk and proximal limbs
- increased risk of organ involvement - renal, lung, cardiac
what investigations/tests are required for systemic sclerosis?
- blood tests - anti-Scl-70 & ANA = diffuse, anti-centromere = limited
- Pulmonary function tests (PFTs) - asses lung involvement
- Echocardiogram: To assess for pulmonary hypertension and cardiac involvement.
- ct scan - organ involvement
- skin biopsy - extent of fibrosis
what is the treatment/management for systemic sclerosis?
- Mycophenolate - immunosupressant
- PPI - reflux
- ACE inhibitors - prevent renal crisis
- Fluoxetine, vasodilators - raynauds
- Cyclophosphamide(im-sup), PDE5 - pulmonary fibrosis
- Annual echocardiograms and pulmonary function tests
- Early detection of pulmonary arterial hypertension
- Autologous stem cell transplant - severe cases
what is Sjögren’s Syndrome?
- autoimmune disorder
- attacks moisture-producing glands, primarily the salivary and lacrimal glands
- dry mouth and eyes
what is the classification of sjogrens syndrome?
- primary - absence of underlying autoimmune disease
- secondary - in conjunction with another autoimmune disease eg SLE, RA, Scleroderma, Primary billiary cirrhosis
what are symptoms of sjogrens syndrome?
dry eyes
dry mouth
dental problems - swollen parotid gland
fatigue and pain
Skin, nose, and vaginal dryness
systemic involvement - increased risk of gluten sensitivity, lymphoma, Renal tubular acidosis
what are investigtaions/tests for sjogrens syndrome?
- blood test - antibodies positive eg ANA, Ro/La, rheumatoid factor, raised immunoglobins
- ultrasound - abnormal salivary glands
- biopsy - Sialadenitis on lip (goldst)
- Schirmer’s test - Measures tear production to evaluate dry eyes.
what is the treatment for sjogrens syndrome
- dry eyes - cyclosporine eye drops, punctal plugs to retain moisture, artificial tears
- dry mouth - Saliva substitutes, pilocarpine/cevimeline to stimulate saliva production.
- Hydroxychloroquine - for fatigue, myalgia, rash
- Corticosteroids/immunosuppressants
what are examples of autoimmune Connective tissue disease?
- Systemic lupus erythematosus
- Systemic sclerosis
- Primary Sjögren’s Syndrome
- Dermatomyositis/Polymyositis
what is Dermatomyositis/Polymyositis?
- both cause muscle weakness (close to trunk)
- dermatomyositis also presents with rash
- can affect lungs
what are characteristic of Dermatomyositis rash?
- violet or dusky red rash
- face, eyelids, chest, knees, elbows, and knuckles
- often with swelling
- Gottron’s papules - scaly rash over knuckles
- Heliotrope rash - rash over eyelids
what investigations/tests are done for Dermatomyositis/Polymyositis?
- Muscle enzymes (Creatine Kinase) - raised
- Electromyography (EMG) - Measures electrical activity in muscles
- Muscle biopsy - shows inflammation/fiber necrosis
- Screen for malignancy (PET-CT) - Malignancy associated antibodies
- Chest X ray, PFTs, High resolution CT - for lung involvement
what is the treatment for Dermatomyositis/Polymyositis?
- steroids (inflammation) - Prednisone
- immunosupressive drugs - eg rituximab, methotrexate
- Hydroxychloroquine - for rash
what are some Myositis‐specific autoantibodies?
MDA5,
NXP2,
TIF1-gamma
ARS
what are some inherited connective tissue disorders?
- Marfan’s
- Ehlers Danlos