A middle aged man presented with nephrotic syndrome (>10 gm protein/24hrs with sev dyslipidemia) with severe thrombocytopenia of almost one year duration. His auto immune profiles are negative, renal biopsy could not be done because of sev thrombocytopenis (Plat 5000). He was started on steroid (Pulse solumedrol follwed by oral prednisone), but there was no improvement. Any suggestion regarding further evaluation and management?
Dear Bhupen,
maybe you can use the following case-reports...
Noel N, Meignin V, Rosenstingl S, Ronco P, Boffa JJ. Nephrotic syndrome associated with immune thrombocytopenia revealing Kimura's disease in a non-Asian male. NDT Plus. 2009;2(6):452-4. http://ckj.oxfordjournals.org/content/2/6/452.full.pdf+html
Voiculescu M, Ionescu C, et al. Nephrotic syndrome, idiopathic thrombocytopenic purpura and systemic lupus erythematous induced by interferon-alpha. J Gastrointestin Liver Dis. http://www.jgld.ro/42000/42000_5.html
Best wishes from Germany
Martin
Good morning
I would recommend to treat the patient with rituximab: 2 infusions of 375 mg/m2 each at one week-interval.
Best regards
Pierre Ronco
Dear Bhupen ,
The association between ITP and nephrotic syndrome seems rare.
I would suggest (because of failure of steroids) a classical therapy with plasma exchange plus fresh plasma.
Best regards
Guy Rostoker
The co-existence of the two diseases is very rare and therefore one can not find any trials of therapy. We have reported a case with co-existence of the two disorders. We treated the patient with Rituximab with good result. Please find the reference below:
Coexistence of immune thrombocytopenic purpura and idiopathic membranous glomerulonephritis successfully treated with rituximab. Pathare A, Alkindi S, Khan S, Riyami D, Farooqi M. Platelets. 2010;21(7):575-7
Dear All,
Patient is responding to Azathioprine 50 mg OD with a pulse therapy of Methylprednisolone. Use of rituximab will be a problematic especially in this part of the country because of non availability and cost.
Thank you all again for guided information.
Kidney biopsy remains the cornerstone of diagnosis and treatment.
Why not try IVIG to raise the platelet count in preparation for elective procedure?
Otherwise empiric use of RTX is probably the best solution if availalble or refundable
Is there any sign pointing to lupus nephritis? Pure membranous LN can present with nephrotic syndrome with a conserved renal function for years and negative serologu. In addition, we have observed good response to treatment even with azathioprine and steroids as you mention.
@ Juan Sir, Sero negative lupus in a middle aged Male patient?? We are planning to do renal biopsy on follow up after stabilization of thrombocytopenia, hope we will get some evidence on renal biopsy.
It is very possible to get lupus in males, and when it does happen, they tend to have seronegativity and can be difficult to treat.
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