scholarly journals Severe ischemic retinopathy in a patient with systemic lupus erythematosus without antiphospholipid syndrome: A case report

2015 ◽  
Vol 29 (2) ◽  
pp. 169-171 ◽  
Author(s):  
El Asri Fouad ◽  
Momen Hanane ◽  
Belmalih Mounir ◽  
Zarrouk Rachid ◽  
Reda Karim ◽  
...  
Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4685-4685
Author(s):  
Yang He ◽  
Weiwen Yang ◽  
Changgeng Ruan

Abstract Abstract 4685 Background: The occurrence of thrombotic thrombocytopenic purpura (TTP) in patients with systemic lupus erythematosus (SLE) is rare TTP frequence. In pregnancy women is increased. TTP with simultaneous SLE, and antiphospholipid syndrome (APS) occurring in the same patient is extremely rare. Case report: We described a 41-year-old Chinese female with TTP in her third trimester (33+4 gestational weeks) of pregnancy who suffered from SLE. and APS at the same time. The clinical features and positive laboratory abnormalities included fever, repeated abortion in first trimester, palpitation, dyspnea, orthopnea, hypertension (180/113mmHg), proteinuria, renal dysfunction, thrombocytopenia (platelets count 12×109/L), hemolytic anemia (Hb 67g/L), positive Coombs test, positive lupus test, positive antinuclear antibody, anti-Sm antibody, anti-insulin antibody, anti-ENA antibody and anti-RNP antibody. The level of plasma ADAMTS13 activity was below 5%, and ADAMTS13 inhibitor was present in the patient's plasma. This patient was treated with a combination of corticosteroids and plasma infusion and plasmapheresis. At 33+5 weeks of gestation she delivered a healthy infant via cesarean section. The patient improved quickly with these therapies. Conclusion: This is a rare TTP case with simultaneous SLE, APS and preeclampsia. Her overlaping clinical manifestations and laboratory abnormalities made the diagnosis very difficult. These data indicate that plasma therapy and corticosteroids can improve the prognosis of TTP associated with SLE and APS. In addition, the termination of pregnancy may be also helpful for controlling the development of the disease. Disclosures: No relevant conflicts of interest to declare.


2013 ◽  
pp. 116-121
Author(s):  
Rocco Manganelli ◽  
Salvatore Iannaccone ◽  
Walter De Simone

Introduction: Primary antiphospholipid syndrome (APS) is a thrombophilic disease that should be suspected in the presence of thrombotic events associated with hematologic abnormalities such as thrombocytopenia and prolongation of the activated partial thromboplastin time. The diagnosis must be confirmed by the demonstration of autoantibodies directed against anionic phospholipids and/or phospholipid-binding proteins. The disease can cause arterial thrombosis in any vascular district, including those of the kidney and central nervous system. Case report: In 2006 a 29-year-old male presented with kidney and brain involvement that was attributed to primary APS. The clinical diagnosis was confirmed by the results of a renal biopsy, which excluded the presence of systemic lupus erythematosus (SLE). The patient remained stable through 32 months of follow-up and then developed a malar rash with deteriorating renal function, decreasing platelet count, and reduced complement levels. Serological studies revealed positivity for ANA (homogeneous pattern), dsDNA, ACA, and beta-2-glycoprotein-1 antibodies. The diagnosis was revised to APS secondary to SLE. Conclusions: A diagnosis of primary APS should not be considered permanent: progression to SLE can occur, in some cases years after the original diagnosis. This case highlights the importance of ongoing follow-up of patients diagnosed with primary APS to detect changes that herald the emergence of SLE.


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