Lupus and Connective Tissue Disease

This may cover a wide range of complex autoimmune conditions mainly looked after by rheumatologist. They include:

Systemic Lupus Erythematosus
Mixed connective tissue disease/ Undifferentiated CTD
Systemic Sclerosis/Scleroderma
Sjogren’s Syndrome
Polymyositis/ Dermatomyositis
Vasculitis

History

Patients may present with a variety of symptoms including:

Photosensitive skin rash/ malar rash
Polyarthritis or polyarthralgia
Serositis- unexplained pleurisy/ pericarditis
Unexplained muscle pain and weakness
Unexplained dyspnoea
Raynaud’s
Unexplained Seizures, focal neurological defects, psychosis
Mouth ulcers
Dry eyes, dry mouth
Hair loss
Recurrent miscarriages or unexplained thrombosis
Constitutional symptoms- unexplained fever or weight loss

Examination

Skin – rash
Swollen joints
Mouth ulcers
Scarring alopecia
Sclerodactyly (tightening and thickening of skin over digits)
Raynaud’s with digital ulceration
Telangiectasia
Pleural or pericardial effusion

Investigations

Haematological	Anaemia, leukopenia or thrombocytopenia
Inflammatory markers	CRP, ESR- may be raised
Immunology: Check immunology only if high index of suspicion. CTD diagnosis is based on history – *Please see* Connective Tissue Disease (ANA) Testing – RefHelp	ANA, anti dsDNA positive, extractable nuclear antigen (ENA) positive
Urine dipstick and renal function	Proteinuria, renal impairment
CK	if concerned about muscle weakness See Myositis page

M.A & H.B/S.R – 29-7-26

When to refer:

Consider referral ONLY in patients with symptoms and signs.

When not to refer:

ANA is positive in 1/20 healthy individuals. A positive ANA is also associated with many non rheumatic diseases such as autoimmune thyroid disease, primary biliary cirrhosis, certain viral infections (EBV, CMV, hepatitis B&C), inflammatory bowel disease and lymphoproliferative diseases.

It is more likely to be positive with age.

If the positive ANA is an isolated finding, then the patient does not need a rheumatology review.
Patients with generalised pain or fatigue that have a positive ANA and a negative dsDNA (with no other signs or symptoms)
Patients with Raynaud’s with weekly positive ANA and a negative dsDNA, ENA and no evidence of digital ischemia/ulcers or other CTD signs and symptoms.