Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome
Macrophage-activating syndrome (MAS) is a rare condition characterized by dysfunctional macrophage activation leading to overproduction of cytokines and phagocytosis of erythrocytes, leukocytes, and platelets. MAS is associated with infectious diseases, malignancies, and autoimmune rheumatic disorde...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2018-01-01
|
| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2018/1961585 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850160108322947072 |
|---|---|
| author | Franchesca Cruz-Pérez Salvador Vilá Grissel Ríos Luis M. Vilá |
| author_facet | Franchesca Cruz-Pérez Salvador Vilá Grissel Ríos Luis M. Vilá |
| author_sort | Franchesca Cruz-Pérez |
| collection | DOAJ |
| description | Macrophage-activating syndrome (MAS) is a rare condition characterized by dysfunctional macrophage activation leading to overproduction of cytokines and phagocytosis of erythrocytes, leukocytes, and platelets. MAS is associated with infectious diseases, malignancies, and autoimmune rheumatic disorders. Herein, we present a 22-year-old Hispanic woman with SLE who was hospitalized because of a three-week history of fever, fatigue, polyarthralgia, nausea, and abdominal pain. Initial laboratories showed severe pancytopenia with marked elevation of liver enzymes and ferritin levels. Bone marrow biopsy revealed macrophages with engulfed erythrocytes consistent with MAS. The patient was treated with high-dose corticosteroids, intravenous immunoglobulins, and cyclosporine 3 mg/kg/day. She had a remarkable clinical response to this therapy. She was continued on cyclosporine, and prednisone dose was gradually decreased to 7.5 mg daily without experiencing recurrent disease. She remained in full clinical remission for 12 months. Our case, together with other reports, suggests that combination therapy with corticosteroids, immunoglobulins, and cyclosporine appears to be effective for patients with SLE-associated MAS. Furthermore, cyclosporine seems to be a good drug for maintenance of remission. |
| format | Article |
| id | doaj-art-b0f3db6972f047be93466b30ba5a4dca |
| institution | OA Journals |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-b0f3db6972f047be93466b30ba5a4dca2025-08-20T02:23:15ZengWileyCase Reports in Rheumatology2090-68892090-68972018-01-01201810.1155/2018/19615851961585Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating SyndromeFranchesca Cruz-Pérez0Salvador Vilá1Grissel Ríos2Luis M. Vilá3Division of Rheumatology, Department of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, PR, USADivision of Rheumatology, Department of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, PR, USADivision of Rheumatology, Department of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, PR, USADivision of Rheumatology, Department of Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, PR, USAMacrophage-activating syndrome (MAS) is a rare condition characterized by dysfunctional macrophage activation leading to overproduction of cytokines and phagocytosis of erythrocytes, leukocytes, and platelets. MAS is associated with infectious diseases, malignancies, and autoimmune rheumatic disorders. Herein, we present a 22-year-old Hispanic woman with SLE who was hospitalized because of a three-week history of fever, fatigue, polyarthralgia, nausea, and abdominal pain. Initial laboratories showed severe pancytopenia with marked elevation of liver enzymes and ferritin levels. Bone marrow biopsy revealed macrophages with engulfed erythrocytes consistent with MAS. The patient was treated with high-dose corticosteroids, intravenous immunoglobulins, and cyclosporine 3 mg/kg/day. She had a remarkable clinical response to this therapy. She was continued on cyclosporine, and prednisone dose was gradually decreased to 7.5 mg daily without experiencing recurrent disease. She remained in full clinical remission for 12 months. Our case, together with other reports, suggests that combination therapy with corticosteroids, immunoglobulins, and cyclosporine appears to be effective for patients with SLE-associated MAS. Furthermore, cyclosporine seems to be a good drug for maintenance of remission.http://dx.doi.org/10.1155/2018/1961585 |
| spellingShingle | Franchesca Cruz-Pérez Salvador Vilá Grissel Ríos Luis M. Vilá Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome Case Reports in Rheumatology |
| title | Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome |
| title_full | Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome |
| title_fullStr | Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome |
| title_full_unstemmed | Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome |
| title_short | Efficacy of Cyclosporine in the Induction and Maintenance of Remission in a Systemic Lupus Erythematosus Patient Presenting with Macrophage-Activating Syndrome |
| title_sort | efficacy of cyclosporine in the induction and maintenance of remission in a systemic lupus erythematosus patient presenting with macrophage activating syndrome |
| url | http://dx.doi.org/10.1155/2018/1961585 |
| work_keys_str_mv | AT franchescacruzperez efficacyofcyclosporineintheinductionandmaintenanceofremissioninasystemiclupuserythematosuspatientpresentingwithmacrophageactivatingsyndrome AT salvadorvila efficacyofcyclosporineintheinductionandmaintenanceofremissioninasystemiclupuserythematosuspatientpresentingwithmacrophageactivatingsyndrome AT grisselrios efficacyofcyclosporineintheinductionandmaintenanceofremissioninasystemiclupuserythematosuspatientpresentingwithmacrophageactivatingsyndrome AT luismvila efficacyofcyclosporineintheinductionandmaintenanceofremissioninasystemiclupuserythematosuspatientpresentingwithmacrophageactivatingsyndrome |