​Lupus / Systemic Lupus Erythematosis (SLE)​

• Lupus (NHS Choices), Lupus erythematosis (PCDS)

Scleroderma

• Scleroderma (PCDS)

Sjogren's syndrome

• Sjögren's syndrome: characterized by dry mouth and eyes

• W>M

• Onset is usually in the 3rd/4th decade, but increases with age

• There is an approximately 20% association with Raynaud’s phenomenon

• Sjogren's syndrome (PCDS)

Systemic sclerosis​

• 2major variants of the disease: generalised and limited (around 60%)

• Limited systemic sclerosis, a multisystem autoimmune disorder affecting connective and vascular tissues

• Usually aged 20-60Y, F>M x5

• 1st symptoms is Raynaud’s phenomenon. In the generalised form of the disease, Raynaud’s phenomenon does not precede skin changes by more than a year; in the limited form of the disease, as for this woman, Raynaud’s may precede other symptoms by many years.

• Skin changes tend to affect the peripheries – particularly the hands, feet and face. Hand changes include sclerodactyly (the fingers are swollen and the skin feels tight restricting movement), calcinosis (calcium deposits in the skin which may extrude from the skin), nailfold changes (enlargement of capillary loops which can be visualised on the nail fold) and telangectases. Eventually, atrophy in the nail pulp causes the nails to become smaller and then the whole of the distal finger to atrophy and shrink. The picture in this question shows typical changes of sclerodactyly.

• Early systemic symptoms include tiredness and malaise, arthralgia, and oesophageal dysmotility resulting in reflux and/or dysphagia. A normochromic anaemia of chronic disease is very common. With the limited form of the disease other organ involvement is less likely, although late-onset pulmonary hypertension may occur 20–30 years after the patient developed Raynaud’s.

• Characteristically, patients with systemic sclerosis have anti-centromere autoantibodies which are present in 70–80% of patients with the limited form of the disease. Antinuclear antibodies are positive in around 90%, but are non-specific for systemic sclerosis. Inflammatory markers (CRP/ESR) usually raised.

• Refer rheumatology for diagnosis/mx.

• Treatment focuses on management of complications, however many patients are also maintained on low dose steroids. Trials of immune modulating treatment are ongoing. Prognosis is very variable depending on the nature and extent of systemic involvement.

Resources

• Connective tissue disorders (RCGP)

• Managing uncertainty in autoimmunity (RCGP)

• Systemic sclerosis (PCDS)

• Connective tissue disorders - an overview (PCDS)