Painful Fingers with Stiffness
Thandi is a 14 year old girl with 3 month history of painful fingers with stiffness. Both hands are affected. She finds it difficult to write in morning lessons, but is better in the afternoons. She has been very 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 (1/360). Her local doctor referred her to general paediatrics suspecting 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.
This may be 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 antibody positive). ESR was raised (35mm/hr) but CRP normal. Urinalysis was negative. Other tests were negative.
She was started on hydroxychloroquine to help joint pain.
She remained well but 3 months later developed more malaise, alopecia and joint pain. Repeat blood tests showed raised ds DNA antibody levels, 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 patient on subsequent visits). The use of cyclophosphamide in pubertal girls has a risk of long term impact on fertility and potentially early menopause; given these concerns there is a trend to consider alternative treatments such as mycophenolate mofetil which has comparable efficacy for disease control in JSLE.
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.