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Case 2
Presented by: Dusan Ferluga
Clinical History
A 45-year-old female patient with a very long clinical history is presented. Diagnosis of juvenile rheumatoid arthritis was established at the age of 13 yrs. During the next 9 years, she was treated intermittently with corticosteroids, gold therapy and nonsteroidal anti-inflammatory drugs. At the age of 22 yrs, she fulfilled ARA criteria for a diagnosis of overlapping systemic lupus erythematosus (SLE) (butterfly rush, arthritis, positive antinuclear antibodies, positive anti-ds DNA at a titer of 0.98, and kidney involvement with proteinuria of 1.1 g/24h, erythrocituria and leukocyturia). The first kidney biopsy was performed at 22 yrs age (see histologic findings). She received cyclophosphamide pulses and corticosteroids. Remission of the most SLE symptoms and a release of laboratory findings were achieved, but in the next year a diagnosis of antiphospholipid syndrome was made due to a recurrent deep vein thrombosis and a high titer of the IgG anti-cardiolipin antibodies. A long-term antithrombotic therapy with varfarine was introduced. Except for suffering of polyarthritic symptoms, she was doing acceptably well during the next 15 years.
At age 28, laboratory investigation revealed in addition to positive antinuclear antibodies positive anti-dsDNA antibodies in low titer of 0.45. At age 39, a skin biopsy revealed positive lupus band test and cyclosporin A was introduced although her urinalysis and serum creatinine were normal. For a short period of time, she became hypertensive, but after a withdrawal of cyclosporin A due to its side effects she remained normotensive throughout the time of clinical observation.
At age 40, antinuclear antibodies (1:640), ENA (HTE1), anti-dsDNA (0.86), IgG anticardiolipin antibodies (>30) were found positive and cyclosporin A was replaced with methotrexate, but shortly stopped because of its side effects. Chloroquine 250 mg per day was introduced and she received in 5 years this drug in a cumulative dose of 450 g. She was feeling fairly well during this time and her joint swelling and pain were reduced. The laboratory parameters of inflammation (SR, CRP) were low. However, at age 45, during the last few months, she complained about fatigue and myalgias. Laboratory testing showed increased serum creatinine 152 mol/L, reduced creatinine clearance 0.4 ml/s, proteinuria of 0.25 – 0.5 g per day, mild erythrocyturia and leukocyturia. ARA criteria for SLE were not fulfilled at the time of her second kidney biopsy.
Fig 1: First kidney biopsy, Fig 2 & 3: second biopsy
Only snapshots available
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CHLOROQUINE-INDUCED RENAL AND SYSTEMIC PHOSPHOLIPIDOSIS MIMICKING FABRY DISEASE
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28.12.2007 |
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