Connective Tissue Diseases Flashcards
What is systemic lupus erythematous? [2]
- A multisystemic autoimmune disease in which autoantibodies are made against a variety of autoantigens which form immune complexes
- Inadequate clearance of immune complexes results in a host of immune responses which cause tissue inflammation and damage
List the signs and symptoms of systemic lupus erythematous under the following headings:
- general features? [4]
- skin? [6]
- musculoskeletal? [2]
- cardiovascular? [2]
- respiratory? [2]
- renal? [2]
- neuropsychiatric? [4]
- General features
- fatigue
- fever
- mouth ulcers
- lymphadenopathy
- Skin
- malar (butterfly) rash
- discoid rash
- photosensitivity
- Raynaud’s phenomenon
- livedo reticularis
- non-scarring alopecia
- Musculoskeletal
- arthralgia
- non-erosive arthritis
- Cardiovascular
- pericarditis
- myocarditis
- Respiratory
- pleurisy
- fibrosing alveolitis
- Renal
- proteinuria
- diffuse proliferative glomerulonephritis
- Neuropsychiatric
- anxiety
- depression
- psychosis
- seizures
What investigations would you carry out on a patient with suspected systemic lupus erythematous? [5]
- Immunology
- ANA
- rheumatoid factor
-
anti-dsDNA
- highly specific (> 99%)
- but less sensitive (70%)
- Monitoring
-
ESR:
- during active disease the CRP is characteristically normal
- a raised CRP may indicate underlying infection
-
complement levels (C3, C4)
- low during active disease
- (formation of complexes leads to consumption of complement)
-
anti-dsDNA titres
- can be used for disease monitoring
- (but note not present in all patients)
-
ESR:
What are the treatment options for systemic lupus (SLE)? [6]
- high-factor sunblock
- NSAIDs
- hydroxychloroquine
- for skin flares, give topical steroids
- azathioprine, methotrexate & mycophenolate
- belimumab (monoclonal antibody)
Sjogren’s syndrome:
- what is it? [2]
- who most commonly gets it? [1]
- results in increased risk of what type of malignancy? [1]
- an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces.
- it may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders
- more common in females
- marked increased risk of lymphoid malignancy
What are the clinical features of Sjogren’s syndrome? [8]
- dry eyes: keratoconjunctivitis sicca
- dry mouth
- vaginal dryness
- arthralgia
- Raynaud’s, myalgia
- sensory polyneuropathy
- recurrent episodes of parotitis
- renal tubular acidosis (usually subclinical)
What investigations would you carry out on a patient with Sjogren’s syndrome? [7]
- rheumatoid factor (RF) (positive in nearly 100%)
- ANA (positive in 70%)
- anti-Ro (SSA) antibodies
- anti-La (SSB) antibodies
- Schirmer’s test
- filter paper near conjunctival sac to measure tear formation
- histology:
- focal lymphocytic infiltration
- hypergammaglobulinaemia, low C4
How do you manage Sjogren’s syndrome? [2]
- artificial saliva and tears
- pilocarpine may stimulate saliva production
What is systemic sclerosis? [1]
a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues
What are the 3 types of systemic sclerosis? [3]
- Limited cutaneous systemic sclerosis
- Diffuse cutaneous systemic sclerosis
- Scleroderma (without internal organ involvement)
What are the 2 types of myositis? [2]
- polymyositis
- dermatomyositis
What is polymyositis? [2]
inflammatory disorder causing symmetrical, proximal muscle weakness which is thought to be a T-cell mediated cytotoxic process directed against muscle fibres with prominent skin manifestations
What is dermatomyositis? [2]
an inflammatory disorder causing symmetrical, proximal muscle weakness and characteristic skin lesions that may be idiopathic or associated with connective tissue disorders or underlying malignancy, that does not have prominent skin manifestations
