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ISSN 0185-3252 (Print)
Revista de la Asociación Médica del Centro Médico ABC

2017, Number 2

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An Med Asoc Med Hosp ABC 2017; 62 (2)

Thrombotic thrombocytopenic purpura associated with lupus nephritis

Jordán-Ríos A, Rodríguez-Lobato LG, Turrent-Carriles A, Zúńiga-Varga J

Language: Spanish
References: 24
Page: 120-126
PDF size: 323.28 Kb.


ABSTRACT

We present the case report of a 34-year-old male with a history of knee arthritis previously treated with corticosteroids, with good response. He complained about generalized edema, dark and foamy urine, malaise and nausea. His lab test results showed thrombocytopenia, reduced renal function, nephrotic range proteinuria and active urine sediment. In the etiologic work-up for nephrotic syndrome, we found hypocomplementemia, positive anti-nuclear antibodies and positive anti-DNAds antibodies. A renal biopsy was performed, in which we found a histopathology compatible with diffuse lupus nephritis (class IV-G A8 C2, as classified by the ISN/RPS) so he was treated with an induction regimen with methylprednisolone pulses (3) and IV cyclophosphamide (1 g). After the treatment, he presented with fever, headache, higher grade anemia and thrombocytopenia, indirect hyperbilirubinemia, low haptoglobins, and a blood smear with schistocytes. The definitive diagnosis was lupus nephritis with TTP. Lupus and TTP present together only in 2% of all lupus patients, and both pathologies have similar symptoms, so the diagnosis can be easily confused. The relationship between these two entities has been previously described, but it is very infrequent, so the natural history, prognosis, and risk factors for the relationship are not known exactly. The suspicion and promptness of the diagnosis are very important for the positive outcome in these patients.


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