A 25 year old woman with rash and raised creatinine

You are doing a week as a general/renal physician in Blantyre, Malawi. A 25 year old woman is referred with a 6 month history of fatigue, joint pain, pleuritic chest pain and facial rash. Three months ago at another hospital she was found to be unwell with a Creatinine of 500 micromol/l, Hb 8.2 g/dl, and urinalysis showed 3+ protein, 3+ blood. She was treated with some tablets (identity unknown) and a subsequent creatinine was 200 and Hb rose to 12 g/dl. Now she is unwell again.

On examination she has a rash around her nose and on her cheeks. She is febrile, 38.2C, and has mild swelling of her left and right MCPJ. She is slim and does not look Cushingoid. BP 155/87. There is no oedema and examination of chest, cardiovascular system and abdomen are normal. She has a platelet count of 50 and Hb 6.2g/dl, Creatinine 430 micromol/l, and urinalysis continues to show 3+ for both protein and blood.

  • What other urgent (not too complicated) test results are important?
  • How would you treat the underlying disease given that she cannot afford a renal biopsy?

Please post comments below. Further info on June 6th. Thanks to Gavin Dreyer for this case.

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4 Responses to A 25 year old woman with rash and raised creatinine

  1. James says:

    CRP
    blood film
    LFTs
    Calcium
    ANA/dsDNA Abs

    Rx
    Corticosteroids, (MP then pred)
    MMF/cyclophos/rituximab – subject to availabilities

  2. Tom says:

    I agree with James’ tests and theoretical management strategy. Might the only achievable regime, given location, be steroids and (maybe only oral) cyclophosphamide?

  3. Bean says:

    If we assume the diagnosis to be lupus nephritis, I would firstly exclude infection and then give her oral prednisolone and cyclophosphamide, perhaps with concomitant antiobiotics. Maybe even oral hydroxychloroquine (regardless of absence of joint disease).

    The outlook is not great. She probably has crescentic class IV so would not go for MMF – as long a term remission as possible is desirable, current trial data do not tell us the answer to which regime is best for this. Furthermore, I’m not sure of the benefits, if any, of MP over oral steroids.

  4. Neil Turner says:

    WHAT HAPPENED NEXT?
    Lupus seems the most likely underlying diagnosis.
    Tests (1) She had a high level of Malaria falciparum parasitaemia. (2) She was HIV non-reactive. Neither infection would do well with immunosuppressive therapy for lupus. The level of HIV positivity in the region is high, but in sick patients, in particular medical inpatients, it is much higher (up to 80%).
    Ideal initial treatment according to WHO should be Artemisinin combination therapy, but it is not generally affordable so Quinine is still most commonly used. She responded to treatment and her Hb rose.
    Treatment beyond this point is indeed difficult, but almost certainly she has aggressive inflammatory disease in view of the creatinine changes, and will need cyclophosphamide. MMF might be considered an alternative, but cost, and perhaps the hardest long term evidence for a young patient with severe disease, would usually militate for the former.

    Could this all have been HIV? SLE-type manifestations may occur in HIV; autoimmune phenomena are common in early disease. Her constellation of typical lupus manifestations would be unusual though. Although you might expect the immunosuppression caused by HIV to suppress lupus, there are several reports in the literature of the two conditions occuring together, probably in early HIV infection but nevertheless posing problems for therapy until the CD4 count has responded to HAART.
    Classic HIV nephropathy usually has a more extreme nephrotic phase. Of course many other renal pathologies can occur in HIV infection.

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