Autoimmune connective tissue diseases Flashcards
What MSK complication can arising from SLE
Osteonecrosis of the femoral head
What hypersensitivity reaction is involved in SLE
Type 3
- Immune complexes are deposited in tissue as they fail to clear.
What drugs are associated with lupus
Procainamide
Isoniazid
Hydralazine
SLE is more prevalent in which populations?
African and Asians
Female> Male (9:1)
Dermatological features of SLE
Photosensitive malar rash
- Nasolabia sparing
Discoid lesions
Non-scarring alopecia
Reynaud’s phenomenon
Livedo reticularis
MSK presentation of SLE
Migratory joint pain (non-erosive arthritis)
- Knees, wrist, PIPJ
Myalgia
The most common heart manifestations of SLE are…
Pericarditis (most common)
Libman Sachs endocarditis ( Nonbacterial thrombotic endocarditis)
The most common renal manifestation of SLE is…
Proliferative glomerulonephritis
Neuropsychiatric manifestations of SLE
CVA
Mood disorder
- Depression, anxiety
Psychosis
Transver myelitis
Neonatal/ congenital lupus can present with what cardiac manifestation?
Complete heart block
- As anti-RO antibodies can cross placenta
The most specific serology for SLE is…
Anti-double stranded DNA antibodies
Anti-smith antibodies (ENA)
Antiphospholipid antibodies seen in SLE
Anticardiolipin
Lupus anticoagulant
The most sensitive serology for SLE is…
Anti-nuclear antibodies (ANA)
- 99% are positive.
What markers can be used to monitor disease activity of SLE
ESR
C3, C4 levels
- Lower in acute phase as they are used uo
Anti-dsDNA titres
Pregnant women with SLE have to be given _____ (what and when) to reduce the risk of pre-eclampsia
Low dose aspirin from week 12.
Respiratory manifestations of SLE
Pleurisy (pleuritic chest pain)
Interstitial fibrosis
First line treatment for non-organ threatening disease in SLE is…
Hydroxychloroquine
First line treatment option for organ involvement in SLE is…
Cyclophosphamide
Complications of sjogren’s syndrome
Eyes
- conjunctivitis
- Corneal ulcers
Oral
- Halitosis
- Dental cavities
- Candida
Vagina
- Vaginitis
- Candidiasis
Extra-glandular presentation of Sjogren’s [5]
Joint pain/ arthritis
Vasculitis
Neuropathy
Interstitial lung disease
Nephritis
Causes of secondary Sjogren’s syndrome
Rheumatoid arthritis
SLE
systemic sclerosis
Polymyositis
Sjogren’s syndrome is associated with a 40-60 fold increase in…
Lymphoid malignancy
Serological diagnosis of Sjogren’s
ANA antibodies (70% sensitivity)
- Anti-Ro
- Anti-La
RF
Hypergammaglobulinaemia
Low C4
Histological diagnosis of Sjogren’s syndrome
Salivary lymph gland biopsy
- Shows focal lymphocytic infiltrates
Which two ENA antibodies are associated with Sjogren’s syndrome
Anti-Ro (SSA)
Anti-La (SSB)
Schirmer’s test ________ is suggestive of insufficient tear production, as seen in Sjogren’s syndrome
<10mm
Management of Sjogren’s syndrome
Artifical saliva and tears
Hydroxychloroquine
Features of CREST syndrome
C- Calcinosis
R- Raynaud’s phenomenon
E- Esophageal dysmotility
S- Sclerodactyly
T- Telangectasia
Types of systemic sclerosis
Limited cutaneous
Diffuse cutaneous
Limited cutaneous sclerosis affects (distal/proximal) region and has a _______ progression
Affects distal regions and has slow progression
What type of systemic sclerosis presents with CREST syndrome
Limited cutaneous sclerosis
Diffuse cutaneous sclerosis affects (distal/proximal) region and has a _______ progression
Affects the proximal region and shows a rapid progression
What type of systemic sclerosis has a high risk of renal crisis
Diffuse systemic sclerosis
Serology diagnosis for diffuse cutaneous sclerosis
ANA antibodies
Anti-scl 70
Anti-RNA polymerase III antibodies
Serology diagnosis for limited cutaneous sclerosis
ANA antibodies
Anti-centromere antibodies
Elevated ___________ is strongly predictive of a _______ in diffuse cutaneous sclerosis
Anti-RNA polymerase III antibodies, strongly predictive of a renal crisis.
Raynaud’s phenomenon can be managed using…
CCB- Nifedipine
ILD in systemic sclerosis can be managed using…
High dose corticosteroid + cyclophosphamide
Scleroderma renal crisis can be managed using…
ACE inhibitor
Types of idiopathic inflammatory myositis
Inclusion body myositis
Dermatomyositis
Polymyositis
Inclusion body myositis presents with…
Asymmetrical proximal limb weakness + exercise intolerance
Diminished deep tendon reflexes
Dysphagia
Inclusion body myositis presents with _________ muscle weakness, whereas polymyositis presents with _______ muscle weakness
IBM= asymmetrical proximal weakness
Polymyositis= symmetrical proximal weakness.
Polymyositis features
Symmetrical muscle weakness
Bulbar and intercostal muscle weakness
Dermatomyositis features
Dermatological
- Photosensitive, V-shaped rash (anterior chest)
- Heliotrope rash on eyelids
Systemic
- Fever, arthalgia, weight loss
Cardiac
- Arrhythmias, DCM
Gottron’s papules
Gottron’s papules are associated with ______
Dermatomyositis
In myositis, an electromyography would show…
Polyphasic action potentions
Myositis will show elevated muscle enzymes, which include…
Creatinine kinase
ALT
LDH
In myositis, serological investigations show elevated…
Anti-nuclear antibodies
Anti-synthase antibodies
Definite diagnosis of myositis involves…
Muscle biopsy and history
Patients diagnosis with myositis have an increased risk of _______ so must be screened
Malignancies
Patients with myositis have an increase risk of which malignancies
- Breast
- Lung
- Ovary
_______ is used for the initial pharmacological management of myositis
Corticosteroids (prednisolone)
Drug causes of erythema nodosum
Penicillin
COCP
Phenytoin
Sulphonamides
Treatment of erythema nodosum
NSAIDs
Investigations for sarcoidosis
Rule out TB
- Mantoux tuberculin test
Bloods
- Bone profile= raised calcium and vit D
- Raised serum ACE
- LFTs- abnormal if liver affected
Serology
- Raised interleukin-2 receptor
ECG
Imaging
- Chest X-ray
- CT
Respiratory function test
Lymph node biopsy and histology
- Non-caseating granuloma with epitheliod cells.
CXR in sarcoidosis will show…
Bilateral hilar lymphadenopathy
What group of people are most commonly affected by sarcoidosis?
Age 20-40
Afro-caribbean
Women
First-line management of sarcoidosis
Oral corticosteroids
Second line management in sarcoidosis
DMARDs
- Methotrexate
- Azathioprine
- Leflunomide
- Mycophenolate
What are the features of Lofgren’s syndrome
Seen in sarcoidosis:
- Erythema nodosum
- Bilateral hilar lymphadenopathy
- Polyarthralgia
What are the features of Heerfordt’s syndrome
Seen in sarcoidosis:
- Submandibular/ parotid gland inflammation
- Uveitis
- CN7 palsy
