Painful Fingers

Neela is a 14 year old girl with 3 month history of painful fingers. Both hands are affected. She finds it difficult to write in morning lessons, but is better in the afternoons. She has been tired and her periods have been irregular.

There is a family history of rheumatoid arthritis (maternal grandfather) and so her mother thought she might have the same. She saw her local doctor and blood tests showed a slightly low lymphocyte count but otherwise normal full blood count, raised ESR (35mm/hr) and ANA (Indirect Immunofluorescence) positive (1 /160). Her local doctor referred her to general paediatrics suspecting Juvenile Idiopathic Arthritis (JIA). She was then referred to paediatric rheumatology.

At her clinic appointment 3 weeks later, she looked pale and tired. Her pGALS assessment demonstrated that she was reluctant to move her fingers and had a reduced full finger tuck to make a fist, but there was no apparent joint swelling. There was no abnormality on chest, heart, abdominal examination.

What is the diagnosis?

This is Juvenile Systemic Lupus Erythematosus (JSLE)

There are features of a multisystem illness but infection and malignancy need to be excluded with blood tests (full blood count, film), acute phase reactants, autoantibodies, complement (C3,C4), thyroid function and urinalysis. She has polyarthralgia predominantly.

Investigations demonstrated features consistent with JSLE (mild lymphopenia, low C3 and C4. ANA positive and ds DNA positive). ESR was raised (35mm/hr) but CRP normal. Urinalysis was negative (.e. renal involvement unlikely).

She was started on hydroxychloroquine to help joint pain.

What are the key issues for management ?

Impact of diagnosis upon the young person and family

Provide disease information to improve understanding; signpost to resources (e.g websites).
Discuss role of specialist team - how and who to contact for advice.

Explanation about disease process and management

Encourage attendance for appointments and explain why review is necessary.
Aim to rationalise appointments if possible to minimise disruption.
Need for potent immunosuppression over prolonged time needing regular admission.
Impact on fertility (males will require consideration of sperm banking prior to cyclophosphamide)
Advice on vaccination schedules.
Risk of thrombosis - need to avoid smoking, avoidance of oestrogen containing pills.

Communication

Contact the extended multidisciplinary team - specialists, primary care, school health.
Liaise with school to improve understanding of condition, treatment and impact.

Symptom management

Tiredness and fatigue; advice about pacing & building up stamina. Family may need support establishing bed times, sleep hygiene & appropriate rest.
Regular pain relief and anti-inflammatory medication (non-steroid anti-inflammatory drugs NSAIDS) may help relieve joint pain.
Impact on menstrual cycle. Consider referral for contraceptive pill although there may be concerns about oestrogen containing pills that may exacerbate disease flares.
Advise about sun safety & use of sun block (likely most days even if not sunny) to reduce photosensitive rash.
Side effects of corticosteroids. Explanation of the need to achieve disease control whilst minimising side effects. Many side effects are reversible; particular support is needed for;

Weight gain - diet, exercise, consider dietician.
Striae - consider oil based topical or other moisturising products. Explain that striae will become less visible over time.
Acne - consider referral to primary care for specific treatment.

Neela's Progress

She remained well but 3 months later developed more malaise, alopecia and joint pain. Repeat blood tests showed increase ds DNA, worse lymphopenia, anaemia and urinalysis showed proteinuria and haematuria. Her blood pressure was normal. She was admitted for assessment and had a renal biopsy which demonstrated glomerulonephritis (WHO Class IV). She was started on IV corticosteroids and cyclophosphamide (to be given over a series of months as a day case on subsequent visits).

Whilst as an inpatient she had considerable input from the nurse and doctor to explain her disease, treatment and advice for when she was discharged home on oral corticosteroids and when to seek medical advice if she developed a fever or was unwell. She had many concerns about her corticosteroids and potential for weight gain. She was much better after her initial treatment and had regular input from the nurses and therapists who liaised with school about her illness, coping with her medication and need for regular appointments.

Painful Fingers

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