inflammatory connective tissue disorders Flashcards

1
Q

What environmental factors can trigger SLE?

A

UV radiation
Oestrogen
Medications (procainamide, hydralazine, isoniazid)

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2
Q

What are the common symptoms of SLE?

A

Fever
Rash
Joint pain

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3
Q

How is SLE diagnosed?

A
4 criteria:
Malar rash (butterfly)
Discoid rash (plaque like)
Photosensitivity 
Ulcers in mucosa of mouth to nose
Serositis (pleuritis, pericarditis)
Renal disorders (DPG)
Neurologic disorders (seizures, psychosis)
Blood- anaemia, thrombocytopenia, leucopenia 
Antinuclear antibody 
Anti-smith/dsDNA/phospholipid
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4
Q

How is fibromyalgia diagnosed?

A

> 7 areas of pain, >5/12 symptom severity (fibrosis fog, fatigue, poor sleep, headache
5 areas pain, >9/12
Symptoms present for 3 months

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5
Q

How is fibromyalgia treated?

A

Exercise
Relaxation
Sleep hygiene
Drugs: amitriptyline, SNRIs, pregabalin, gabapentin

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6
Q

What are the symptoms fo polymyalgia rheumatica?

A

Pains starts on one side and progresses to both in weeks
More severe at morning and night and longer than an hour, improves after activity
Low grade fever
Loss of appetite
Temporal arthritis (headaches, vision problems)

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7
Q

How is fibromyalgia rheumatica diagnosed?

A

High ESR and CRP

Normal creatinine kinase

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8
Q

How is amyloidosis diagnosed?

A

Biopsy of involved organ
Fat pad biopsy
Dye: congo red makes amyloid pink (apple green under polarised light)

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9
Q

What is Sjogren’s syndrome?

A

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, where it usually develops around 10 years after the initial onset. Sjogren’s syndrome is much more common in females (ratio 9:1). There is a marked increased risk of lymphoid malignancy (40-60 fold).

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10
Q

What are the features of sjorgren’s syndrome?

A
dry eyes: keratoconjunctivitis sicca
dry mouth
vaginal dryness
arthralgia
Raynaud's, myalgia
sensory polyneuropathy
recurrent episodes of parotitis
renal tubular acidosis (usually subclinical)
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11
Q

What are the investigations for sjorgren’s syndrome?

A

rheumatoid factor (RF) positive in nearly 100% of patients
ANA positive in 70%
anti-Ro (SSA) antibodies in 70% of patients with PSS
anti-La (SSB) antibodies in 30% of patients with PSS
Schirmer’s test: filter paper near conjunctival sac to measure tear formation
histology: focal lymphocytic infiltration
also: hypergammaglobulinaemia, low C4

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12
Q

What are antihistoen antobodies associated with?

A

Drug induced SLE

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13
Q

What is seen on investigations in SLE?

A

Normochromic, normocytic anaemia
Neutropenia/lymphopenia and thrombocytopenia
ESR raised
Anti-dsDNA

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14
Q

What is systemic sclerosis (scleroderma)?

A

Skin disease with Raynaud’s
Most common in middle aged women
Thickening of vascular walls and narrowing of lumen

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15
Q

How is scleroderma diagnosed?

A

normochromic,normocytic anaemia
Raised ESR
Anti-RNA polymerase
Anti-topoisomerase

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16
Q

What is Sjogren’s syndrome?

A

Destruction of epithelial exocrine glands, especially lacrimal and savilary
Most common in middle aged women

17
Q

What are the clinical features fo Sjogren’s?

A
Dry eyes and mouth 
Can be associated with SLE or RA
Raynaud's phenomenon 
Renal tubular defects 
Pulmonary fibrosis 
Vasculitis
Non-Hodgkins B cell lymphoma
18
Q

How is Sjogren’s diagnosed?

A

Antinuclear, anti-ro and RA
Lymphocyte infiltration and destruction of acing tissue
Positive Schirmer test- defective tear production

19
Q

What are the vascular features of SLE?

A

Raynaud’s phenomenon
Vasculitic rash
Venous thrombosis

20
Q

What are the clinical features of scleroderma?

A
Sclerodactyly (thickening and tightness of skin 
Raynaud's phenomenon 
Abnormalities of nail bed (splinter haemorrhages)
Arthralgias, myalgia 
Dyspepsia 
Pulmonary artery hypertension 
, interstitial lung disease (2/3 deaths)
Pericardial/myocardial disease
21
Q

What are the clinical features of SLE?

A

Vasculitis and rash- ulceration, malar, photosensitivity
Arthralgias
Anaemia
Serositis (pleurisy, pericarditis)
Glomerulonephritis
Seizures, psychosis
Jaccoud’s arthropathy (swan neck deformities on several digits)