inflammatory connective tissue disorders Flashcards
What environmental factors can trigger SLE?
UV radiation
Oestrogen
Medications (procainamide, hydralazine, isoniazid)
What are the common symptoms of SLE?
Fever
Rash
Joint pain
How is SLE diagnosed?
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
How is fibromyalgia diagnosed?
> 7 areas of pain, >5/12 symptom severity (fibrosis fog, fatigue, poor sleep, headache
5 areas pain, >9/12
Symptoms present for 3 months
How is fibromyalgia treated?
Exercise
Relaxation
Sleep hygiene
Drugs: amitriptyline, SNRIs, pregabalin, gabapentin
What are the symptoms fo polymyalgia rheumatica?
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)
How is fibromyalgia rheumatica diagnosed?
High ESR and CRP
Normal creatinine kinase
How is amyloidosis diagnosed?
Biopsy of involved organ
Fat pad biopsy
Dye: congo red makes amyloid pink (apple green under polarised light)
What is Sjogren’s syndrome?
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).
What are the features of sjorgren’s syndrome?
dry eyes: keratoconjunctivitis sicca dry mouth vaginal dryness arthralgia Raynaud's, myalgia sensory polyneuropathy recurrent episodes of parotitis renal tubular acidosis (usually subclinical)
What are the investigations for sjorgren’s syndrome?
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
What are antihistoen antobodies associated with?
Drug induced SLE
What is seen on investigations in SLE?
Normochromic, normocytic anaemia
Neutropenia/lymphopenia and thrombocytopenia
ESR raised
Anti-dsDNA
What is systemic sclerosis (scleroderma)?
Skin disease with Raynaud’s
Most common in middle aged women
Thickening of vascular walls and narrowing of lumen
How is scleroderma diagnosed?
normochromic,normocytic anaemia
Raised ESR
Anti-RNA polymerase
Anti-topoisomerase