Rheumatology

Question 1

Felty syndrome

Answer 1

An extra-articular manifestation of seropositive rheumatoid arthritis

Key features:
Rheumatoid arthritis + neutropenia + splenomegaly

Question 2

Still’s disease

Answer 2

Typically a form of JIA, can rarely present in adulthood

Features:
- Fever
- Maculopapular rash
- Myalgia
- Generalised lymphadenopathy

More rarely:
- Hepatosplenomegaly
- Pericarditis
- Pleurisy

Investigations:
- Raised WCC, CRP, ESR
- Seronegative - incl. normal RF

Question 3

Sjogren’s syndrome

Answer 3

Autoimmune destruction of secretory glands by lymphocytes and plasma cells

Features:
- Dry eyes and mouth
- Other dry membranes e.g. nasal, vaginal, skin
- Fatigue
- Arthralgia
- Dysphagia
- Otitis media

Investigations:
- Autoantibodies:
- ANA and anti-Ro found in 70%
- RF found in 50%
- Anti-La found in 30%
- Schirmer test
- Salivary gland biopsy

Management:
- Sicca symptoms - artificial saliva/tears
- Consider steroids/DMARDs/hydroxycholoquine if have severe systemic manifestations

Question 4

Goodpasture’s disease

Answer 4

Aetiology:
- HLA-DRB1*15:01 and HLA-DR15
- Leads to formation of antibodies against alpha-3 chain of type IV collagen
- M&raquo_space; F

Features:
- Pulmonary haemorrhage - haemoptysis
- Anti-alveolar basement membrane
- Glomerulonephritis with haematuria, proteinuria
- Peripheral oedema
- Dyspnea

Investigations:
- Renal impairment
- Normal ESR
- Anti-GBM serology
- CXR - bilateral patchy infiltrates
- Renal biopsy - crescenteric glomerulonephritis with linear IgG staining

Management:
- Plasma exchange + immunosuppression (steroids + cyclophosphamide)

Question 5

Systemic sclerosis

Answer 5

A connective tissue disorder characterised by skin fibrosis/thickening, involvement of internal organs, and vascular damage
- Classified as limited or diffuse according to
skin involvement
- Fibrosis affects skin, GI tract, heart
- Vascular damage leads to pulmonary HTN,
renal disease, Raynaud’s

Features:
- Skin:
- Initial oedematous phase, then hidebound
phase
- Pigmentation and hair loss
- Raynaud’s
- Can progress to digital ulcers and calcinosis
- MSK:
- Arthralgia
- Erosive arthritis in 30%
- Myositis
- Flexion deformities
- Renal
- Renal crisis - malignant HTN, rapid renal
impairment, ‘onion skin’ intrarenal
vasculature
- Pulmonary
- Pulmonary fibrosis
- Pulmonary HTN
- Gastrointestinal
- Impaired motility
- Oesophageal reflux and strictures
- Gastric dilatation
- Bacterial overgrowth
- Constipation

Autoantibodies:
- ANA in 90%
- Anti-centromere = 90% of limited
- Anti-Scl70 = 20-40% of diffuse

Management:
- Screening and symptom support
- Raynaud’s - vasodilators
- Rapid progressive scleroderma - mycophenolate
- Renal crisis - ACEi
- Pulmonary fibrosis - cyclophosphamide, steroids
- Pulmonary HTN - iloprost, bosentan

Question 6

Types of systemic sclerosis

Answer 6

Limited scleroderma with systemic involvement
- Scleroderma limited to face, neck, distal limbs
- Anti-centromere +ve
- Presents with Raynaud’s
- CREST features
- Pulmonary HTN

Diffuse scleroderma with limited involvement
- Scleroderma including trunk, face, entire limbs
- Anti-Scl70 in 20-40%
- Presents with arthritis and dactylitis
- Renal crises