Connective Tissue Disease and Vasculitis Flashcards
1
Q
What are the features of polymyositis/dermatomyositis?
A
- Chronic inflamamtion of striated muscle (with rash in case of dermatomyositis)
- Progressive muscle weakness +/- pain, fatigue, interstitial alveolitis or fibrosis of the lungs
2
Q
What are the diagnostic features of polymyositis/dermatomyositis?
A
- Key investigation in diagnosing myositis is creatine kinase (CK) (normally less than 300U/L but can be over 1000 in myoitis)
- Painful proximal myopathy with weakness - occurs bilaterally, mostly affects shoulder and pelvic girdle, develops over weeks
- Evidence of inflammation in the muscle (biopsy/MRI)
- Elevation of other muscle enzymes (ALT, LDH)
- Characteristic EMG pattern
- Characteristic JDM rash (in case of dermatomyositis)
- Anti-Jo-1 antibodies are present in polymyositis and dermatomyositis
- Anti-Mi-2 antibodies and ANA are present in dermatomyositis
3
Q
What are the features of SLE?
A
- Butterfly rash on face that spaces nose (photosensitive)
- Mucosal ulceration (painless)
- Alopecia
- Skin vasculitis
- Capillary dilatation
- Fatigue
- Weight loss
- Arthralgia and non-erosive arthritis
- Lymphadenopathy
- SOB
- Pleuritic chest pain
- Hair loss
- Raynaud’s phenomenon
4
Q
How is SLE diagnosed?
A
- 4/11 of the SLI classification criteria (including ANA)
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Serositis
- Renal disorder
- Neurological disorder
- Haematological disorder
- Immunological disorder
- ANA
- Anti-dsDNA is specific to SLE - also good for monitoring
- Anti-Smith antibodies are highly specific to SLE but not very sensitive
- Other investigations
- FBC
- C3 and C4 levels
- CRP
- ESR
- Immunoglobulins
- Urinalysis for urine protein:creatinine for proteinuria lupus nephritis
5
Q
How is SLE treated?
A
- Anti-inflammatory medication and immunosuppression
- First line:
- NSAIDs
- Steroids
- Hydroxychloroquine
- High SPF sunblock
- Other immunosuppresants:
- Methotrexate
- Mycophenolate mofetil
- Azathioprine
- Tacrolimus
- Leflunomide
- Ciclosporin
- Biologic therapies for patients with severe disease
- Rituximab (targets CD20)
- Belimumab (targets B-cell activating factor)
6
Q
What are the features of Sjorgren Syndrome?
A
- Autoimmune condition of the exocrine glands - leads to symptoms of dry mucous membranes
- Primary (in isolation) or secondary (with SLE or RA)
- Dry eyes, dry mouth, salivary gland enlarged and tender, and dry mucosal membranes
- Arthralgia, Raynaud’s, neurological neuropathy, kidneys and lungs
- Associated with lymphoma
7
Q
What are the features of Giant Cell Arteritis (large vessel vasculitis)?
A
- Very common in elderly
- Features include:
- Severe unilateral headache typically around temple and forehead
- Scalp tenderness ‘when brushing hair’
- Jaw claudications
- Blurred or double vision
- Irreversible painless complete sight loss can occur rapidly
- Fever
- Muscle aches
- Weight loss
- Loss of appetite
- Peripheral oedema
- Related to polymyalgia rheumatica with presents with pain and stiffness in muscles of neck and shoulder girdle
8
Q
What are the features of Takayasu arteritis (large vessel vasculitis)?
A
- Younger women (15-25yrs)
- Vascular insufficiency in aorta and large tributaries
- Diagnoses involves imaging antiography and CT
9
Q
What are the features of polyarteritis nodosa (medium vessel vasculitis)?
A
- Associated with HBV, HCV and HIV
- Fever and weight loss typical but not specific
- Organ infarction or ischaemia typically include gut, brain, heart, liver, skin, PNS, testis, limbs and kidneys
- Also associated with rash called livedo reticularis (mottled, purplish, lace like rash)
10
Q
What are the features of Kawasaki disease (medium vessel vasculitis)? (rememer CRASH & Burn)
A
- Children only (usually under 5 years)
- CRASH & Burn
- Conjunctivitis (bilateral)
- Rash (erythematous)
- Adenopathy (cervical, commonly unilateral)
- Strawberry tongue (red tongue with prominent papillae)
- Hands (erythema and desquamation (skin peeling) of palms and soles)
- Burn (persistent high fever >5 days)
- Aneurysm formation in medium to large sized arteries is a key complication
- Treat with aspirin and IVIG
11
Q
What are the features of HSP deposit IgA (immune complex mediated, small vessel vasculitis)?
A
- Commonest vasculitis in childhood (usually presents in under 10 years)
- Often triggered by upper airway infection or gastroenteritis
- Classic featrures include:
- Palpable purpura
- Abdominal pain/rash
- Joint pain
- Renal involvement
- Management is typically supportive
12
Q
What are the features of microscopic polyangitis (ANCA associated, small vessel vasculitis)?
A
- Perinuclear p-ANCA Myeloperoxidase (MPO) positive
- Five most common manifestations are kidney GN, weight loss, skin lesions, peripheral neuropathy/mononeuritis multiplex and fevers
13
Q
What are the features of granulomatosis with polyangitis or Wegener’s granulomatosis (ANCA associated, small vessel vasculitis)?
A
- Cytoplasmic c-ANCA Proteinase 3 (PR3) positive
- Classically involves upper/lower respiratory tract and kidneys
- Nose bleeds
- Saddle shaped nose due to perforated nasal septum
- Cough
- Wheeze
- Haemoptysis
- Can cause RPGN in the kidneys
14
Q
What are the features of eosinophilic granulomatosis with polyangitis (ANCA associated, small vessel vasculitis)?
A
- Perinuclear p-ANCA Myeloperoxidase (MPO) positive
- Asthma associated disease
- Eosinophils everywhere
- Peripheral neuropathy
15
Q
How is GCA diagnosed?
A
- Clinical presentation
- Raised ESR (usually 50mm/hr or more)
- Temporal artery biopsy findings
- Multinucleated giant cells are found
- Can also include FBC (normocytic anaemia and thrombocytosis) LFTs (raised ALP), CRP (raised) and duplex US (hypoechoic halo sign)
16
Q
Management of GCA
A
- Start steroids immediately due to risk of permanent sight loss - 40-60mg prednisolone per day
- Aspirin decreases visual loss and strokes
- PPI for gastric protection while on steroids
- May need to refer to vascuar surgeons for biopsy, rheumatology for specialist diagnosis and ophthalmology for review if they develop visual symptoims
- Ongoing management - will need steroids until symptoms resolve, then slowly wean steroids
17
Q
Complications of GCA
A
- Early neuro-ophthalamic complications
- Vision loss
- CVA
- Late
- Relapse of the condition
- Steroid related side effects and complications
- CVA
- Aortitis leading to aortic aneurysm and aortic dissection
18
Q
Definition of PMR
A
- Inflammatory condition that causes pain and stiffness in the shoulders, pelvic girdle and neck
- Usually affects those >50 years
- More common in women and caucasians
19
Q
Features of PMR
A
- Core features present for >2 weeks
- Bilateral shoulder pain that may radiate to the elbow
- Bilateral pelvic girdle pain
- Worse with movement
- Interferes with sleep
- Stiffness for at least 45 minutes in the morning
- May also have weight loss, fatigue, low grade fever, low mood, upper arm tenderness, carpal tunnel syndrome and pitting oedema
20
Q
Diagnosis of PMR
A
- Clinical presentation and response to steroids
- Inflammatory markers usually raised but if not does not exclude
- Can add other investigation to exclude other conditions
21
Q
Management of PMR
A
- 15mg prednisolone per day
- After 1 week reassess - should expect improvement, if not likely not PMR
- After 3-4 weeks reassess - expect 70% improvement in symptoms, inflammatory markers return to normal
- If good response then:
- 15mg until symptoms fully controlled
- 12.5mg for 3 weeks
- 10mg for 4-6 weeks
- Reduce by 1mf every 4-8 weeks
- If symptoms recurr then increase dose - if still on at 2 years refer to rheumatology
22
Q
Features of cutaneous leukocytoclastic vasculitis
A
- Palpable purpuric rash
- Arthralgia
- GN
- Caused by HCV and drugs (sulphonamides, penicillin)
23
Q
Features of Cryoglobulinaemia
A
- Simple
- Monoclonal IgM
- Secondary tomyeloma/CLL/Waldenstroms
- Hyperviscosity
- Visual disturbance
- Bleeding from mucous membranes
- Thrombosis
- Headache, seizures
- Mixed (80%)
- Polyclonal IgM
- Secondary to SLE, Sjogrens, HCV, mycoplasma
- Immune complex disease
- CN
- Palpable purpura
- Arthralgia
- Peripheral neuropathy
24
Q
Features of Goodpastures Syndrome
A
- Anti-GBM antibodies
- RPGN
- Haemoptysis
- CXR shows bilateral zone infiltrates (haemorrhage)
- Treat with immunosuppression and plasmapheresis
25
Q
Complications of SLE
A
- CV disease
- Infection
- Anaemia of chronic disease
- Pericarditis
- Pleuritis
- Interstitial lung disease
- Lupus nephritis
- Neuropsychiatric SLE (optic neuritis, psychosis, transverse myelitis)
- Recurrent miscarriage
- VTE
26
Q
Mnagement of dermatomyositis/polymyositis
A
- Corticosteroids are first line in both conditions
- Can also use immunisuppresants (azathioprine), IVIG and biological therapies (infliximab or enteracept)
27
Q
Schirmer Test for Sjogren’s Syndrome
A
- The Schirmer test involves inserting a folded piece of filter paper under the lower eyelid with a strip hanging out over the eyelid. This is left in for 5 minutes and the distance along the strip hanging out that becomes moist is measured. The tears should travel 15mm in a healthy young adult. A result of less than 10mm is significant.
28
Q
Management of Sjogren’s Syndrome
A
- Artificial tears
- Artificial saliva
- Vaginal lubricants
- Hydroychloroquine is used to halt disease progression
29
Q
Complications of Sjogren’s syndrome
A
- Eye infections (conjunctivitis, corneal ulcers)
- Oral problems (dental cavities, candida infection)
- Vaginal problems (candidiasis, seual dysfunction)
