[Thrombotic thrombocytopenic purpura - a rare cause of thrombocytopenia in systemic lupus erythematosus].
METHODS
An 18-year old woman with a systemic lupus erythematosus was referred to our hospital with anaemia, thrombocytopenia and neurological symptoms. Skin haemorrhages had occurred some weeks before. The patient also noticed cardiac palpitations. After the primary care doctor had increased the dosage of prednisolone from 7.5 mg per day to 20 mg per day the symptoms didn't substantially change and that the patient was referred to hospital. An acute lupus flare was assumed.


METHODS
Laboratory examinations showed a Coombs-negative haemolytic anaemia with schistocytes. Von Willebrand factor(vWF)-cleaving protease was decreased. The presence of an inhibitor of the enzyme was proved. Further test results as anti-ds-DNA, anti-Sm, C 3, ESR, CRP were normal. C 4 was slightly decreased. Cerebral MRI, cerebral SPECT and Duplex sonography of the extracranial arteries didn't show any pathologic findings. The performed MRI of the heart revealed signs of myocardial damage.


METHODS
As cause of the recent symptoms a thrombotic-thrombocytopenic purpura (TTP) was diagnosed. This obviously was due to an auto-antibody against the vWF-cleaving protease. After intensive immunosuppressive therapy with prednisolone and cyclosporin the clinical and laboratory findings normalised. An acute therapy with fresh frozen plasma and plasmapheresis was not necessary, as the symptoms were moderate. Because of the transient cerebral deficits for a short time we administered a low dose of aspirin. The MRI signs of myocardial affection didn't change after 3 months of immunosuppressive treatment so that we started an ACE inhibitor treatment.


CONCLUSIONS
Thrombocytopenia in systemic lupus erythematosus demands an individual differential diagnosis. Rare causes as TTP have to be excluded. This disorder has to be diagnosed quickly and the adequate therapy has to be started early.
Natusch
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A
Rheumaklinik Berlin-Buch, Germany. a.natusch@immanuel.de
Gromnica-Ihle
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E
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology; D003524:Cyclosporins / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008666:Metalloendopeptidases / Q000037:antagonists & inhibitors* / Q000276:immunology; D011239:Prednisolone / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D015899:Tomography, Emission-Computed, Single-Photon; D018616:Ultrasonography, Doppler, Duplex
D000806:Angiotensin-Converting Enzyme Inhibitors; D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D003524:Cyclosporins; D007166:Immunosuppressive Agents; D011239:Prednisolone; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human