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Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model.

Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO - Pediatr Rheumatol Online J (2008)

Bottom Line: We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear.Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM.The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pediatrics, Division of Pediatric Rheumatology, Ohio State University College of Medicine, Columbus, Ohio, USA. rennebohmr@pediatrics.ohio-state.edu.

ABSTRACT
We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear. Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM. The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.

No MeSH data available.


Related in: MedlinePlus

Immunosuppressive therapy. Graphic depiction of the patient's immunosuppressive treatment.
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Figure 3: Immunosuppressive therapy. Graphic depiction of the patient's immunosuppressive treatment.

Mentions: She was aggressively treated as shown in Figure 3. Initially, she received high dose oral prednisone, frequent pulses of methylprednisolone, monthly IVIG, and monthly pulses of IV cyclophosphamide. After receiving 6 monthly pulses of cyclophosphamide, she was placed on mycophenylate mofetil (MM) for maintenance therapy. However, since she was unable to tolerate side effects of MM (malaise and nausea), pulse cyclophosphamide therapy was resumed. Not shown in Figure 3 is a single dose of natalizumab that she received during the first week of treatment. Shortly after that single dose, we decided that the risk/benefit ratio for natalizumab did not justify further doses. Since then, we have not used natalizumab for this patient or any subsequent SS patients.


Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model.

Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO - Pediatr Rheumatol Online J (2008)

Immunosuppressive therapy. Graphic depiction of the patient's immunosuppressive treatment.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2267466&req=5

Figure 3: Immunosuppressive therapy. Graphic depiction of the patient's immunosuppressive treatment.
Mentions: She was aggressively treated as shown in Figure 3. Initially, she received high dose oral prednisone, frequent pulses of methylprednisolone, monthly IVIG, and monthly pulses of IV cyclophosphamide. After receiving 6 monthly pulses of cyclophosphamide, she was placed on mycophenylate mofetil (MM) for maintenance therapy. However, since she was unable to tolerate side effects of MM (malaise and nausea), pulse cyclophosphamide therapy was resumed. Not shown in Figure 3 is a single dose of natalizumab that she received during the first week of treatment. Shortly after that single dose, we decided that the risk/benefit ratio for natalizumab did not justify further doses. Since then, we have not used natalizumab for this patient or any subsequent SS patients.

Bottom Line: We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear.Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM.The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pediatrics, Division of Pediatric Rheumatology, Ohio State University College of Medicine, Columbus, Ohio, USA. rennebohmr@pediatrics.ohio-state.edu.

ABSTRACT
We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear. Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM. The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.

No MeSH data available.


Related in: MedlinePlus