1. Six cases of Lupus Presented by Richard A. Furie, M. D. at the November ACR meeting in San Diego

2. Case 1  27 yr old female with a 5 year hx of SLE admitted with confusion and fever. Two months prior had a flare with anemia and pericarditis, hypocomplementemia and elevated anti-DNA antibodies that responded to 40 prednisone. Presently on 15mg/d.

3. Case 1  VS BP120/70, T 104  Lower extremity petechiae  Delirious, disoriented, hyperreflexic with abnormal Babinski’s bilat.  VS BP120/70, T 104  Lower extremity petechiae  Delirious, disoriented, hyperreflexic with abnormal Babinski’s bilat.

4. Case 1  Lab: WBC 15.7 left shift  Hgb 6.8 Gm/dL  Plt 12,000  Creatinine 1.9; LFT’s normal  UA 20 rbc, trace protein;CSF neg  ANA 1/160 homogeneous. anti-DNA positive  C3/C4 normal  Lab: WBC 15.7 left shift  Hgb 6.8 Gm/dL  Plt 12,000  Creatinine 1.9; LFT’s normal  UA 20 rbc, trace protein;CSF neg  ANA 1/160 homogeneous. anti-DNA positive  C3/C4 normal

5. Case 1  Rx: antibiotics, pulse steroids, IVIG not helpful  Smear schistocytes; LDH 1200; Coombs negative; haptoglobin low  Dx TTP;  Rx plasmapheresis or exchange  Rx: antibiotics, pulse steroids, IVIG not helpful  Smear schistocytes; LDH 1200; Coombs negative; haptoglobin low  Dx TTP;  Rx plasmapheresis or exchange

6. Case 1  Thrombotic microangiopathy -TTP,HUS, pregnancy, drugs (cyclosporine,mitomycin), SLE, antiphospholipid syndrome with catastrophic APL syndrome  Autoantibodies to ADAMTS13, the metalloprotease that cleaves von Willebrand factor  Thrombotic microangiopathy -TTP,HUS, pregnancy, drugs (cyclosporine,mitomycin), SLE, antiphospholipid syndrome with catastrophic APL syndrome  Autoantibodies to ADAMTS13, the metalloprotease that cleaves von Willebrand factor

7. Case 2  24 yo female with steroid responsive thrombocytopenia referred for eval of SLE  Physical exam revealed livido reticularis and chorea of the right arm and face  Platelet ct - 125,000; ANA 1/160; PTT 39 sec; Lupus anticoagulant positive; anticardiolipin positive; anti DNA negative; ASO negative  24 yo female with steroid responsive thrombocytopenia referred for eval of SLE  Physical exam revealed livido reticularis and chorea of the right arm and face  Platelet ct - 125,000; ANA 1/160; PTT 39 sec; Lupus anticoagulant positive; anticardiolipin positive; anti DNA negative; ASO negative

8. Case 2  Brain MRI and EEG negative; PET scan showed hot spot in left parietal area.  Spontaneous remission of chorea and PET abn.  One year later she had a similar event on other side for one month with remission.  Brain MRI and EEG negative; PET scan showed hot spot in left parietal area.  Spontaneous remission of chorea and PET abn.  One year later she had a similar event on other side for one month with remission.

9. Case 2  Chorea is described in SLE, Antiphospholipid syndrome and others.  This case favors a stimulatory mechanism, not thrombosis.  Chorea is described in SLE, Antiphospholipid syndrome and others.  This case favors a stimulatory mechanism, not thrombosis.

10. Case 3  27 yr old female with a 5 year hx of SLE. For three weeks had been on on 60 mg/d of prednisone for nephritis.  She stepped up on a bus and ruptured both patellar tendons.  Tendon rupture occurs as a complication of SLE and steroids. Usually little or no inflammation.  27 yr old female with a 5 year hx of SLE. For three weeks had been on on 60 mg/d of prednisone for nephritis.  She stepped up on a bus and ruptured both patellar tendons.  Tendon rupture occurs as a complication of SLE and steroids. Usually little or no inflammation.

11. Case 4  44 yo female with 9 year hx of SLE never on steroids or immunosuppressives despite arthritis and nephritis.  Admitted for acute septic shock.  Skin showed scattered raised violaceous papules with necrotic centers and ischemic toes suggesting Waterhouse-Friderichsen syndrome.  44 yo female with 9 year hx of SLE never on steroids or immunosuppressives despite arthritis and nephritis.  Admitted for acute septic shock.  Skin showed scattered raised violaceous papules with necrotic centers and ischemic toes suggesting Waterhouse-Friderichsen syndrome.

12. Case 4  Lab: WBC 58,000 (91% PMN’s); Hgb 13; Plt 127,000.  Smear - Howell-Jolly bodies.  Blood cultures: Strept pneumoniae  Spleen scan - no function; CT scan - normal sized spleen.  Post recovery uptake in spleen returned to normal and the H-J bodies disappeared.  Lab: WBC 58,000 (91% PMN’s); Hgb 13; Plt 127,000.  Smear - Howell-Jolly bodies.  Blood cultures: Strept pneumoniae  Spleen scan - no function; CT scan - normal sized spleen.  Post recovery uptake in spleen returned to normal and the H-J bodies disappeared.

13. Case 4  Dx; functional asplenia - usually a fatal complication with S. pneumonia or Salmonella sepsis.  This is first case of survival (1990).  Described also in APL and celiac sprue  Underlines the need to fully vaccinate SLE patients.  Dx; functional asplenia - usually a fatal complication with S. pneumonia or Salmonella sepsis.  This is first case of survival (1990).  Described also in APL and celiac sprue  Underlines the need to fully vaccinate SLE patients.

14. Case 5  47 yo with 5 year hx of severe discoid lupus having failed steroids, azathioprine, MTX, hydroxychloroquine.  Great response to Thalidomide.  Inhibition of angiogenesis, TNF, IL-8, COX-2, IL- 6 and IL-10  Somnolence, rash, leukopenia, orthostatic hypotension, sensory neuropathy,constipation, birth defects.  47 yo with 5 year hx of severe discoid lupus having failed steroids, azathioprine, MTX, hydroxychloroquine.  Great response to Thalidomide.  Inhibition of angiogenesis, TNF, IL-8, COX-2, IL- 6 and IL-10  Somnolence, rash, leukopenia, orthostatic hypotension, sensory neuropathy,constipation, birth defects.

15. Case 6  22 yr old with a 3 year hx of SLE and 13 grams of proteinuria. Kidney Bx class IV inflammation, but little chronicity. Received pulse steroids, refused cytoxan but took Mycophenolate mofetil (Cellcept) and enalapril. Failed that and was given Cyclophosphamide without benefit.

16. Case 6 DRUGS Target Phase RiquentAutoAblll Lymphostat BB cellsll RituxanB cellsll/lll Anti-CD 40T cellsthrombosis CTLA41IgT-B cellsll CellCeptLymphlll EculizumabC’l Ablation;Transpla ntation WBCll EpratuzumabB cellslll Anti-IL-6 RCy Rl

17. Case 6 Toulizumab IL-6 inhibitor