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A Case Report of Successful Treatment of Recalcitrant Childhood Localized Scleroderma with Infliximab and Leflunomide.

Ferguson ID, Weiser P, Torok KS - Open Rheumatol J (2015)

Bottom Line: Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide.The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global assessments.Larger longitudinal studies or case series are needed to confirm and further investigate infliximab's role in localized scleroderma.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Division of Rheumatology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.

ABSTRACT
Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide. The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global assessments. Infliximab is a potential treatment option for pediatric localized scleroderma patients who have progression of disease or who are unable to tolerate the side effect profile of more standard systemic therapy. Larger longitudinal studies or case series are needed to confirm and further investigate infliximab's role in localized scleroderma.

No MeSH data available.


Related in: MedlinePlus

Timeline of medical therapy. A graphic summary of the past 5 years of treatment for the patient reported in the case. Topicalcorticosteroids (Top CS), Penicillin VK (Pen VK), Hydroxychloroquine (HCQ), Prednisone (Pred), Methotrexate (MTX), Minocycline(MNC), Mycophenolate mofetil (MMF), Infliximab (IFX), Leflunomide (LEF), IV Methylprednisolone (MP) and No treatment (No Tx).
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Figure 1: Timeline of medical therapy. A graphic summary of the past 5 years of treatment for the patient reported in the case. Topicalcorticosteroids (Top CS), Penicillin VK (Pen VK), Hydroxychloroquine (HCQ), Prednisone (Pred), Methotrexate (MTX), Minocycline(MNC), Mycophenolate mofetil (MMF), Infliximab (IFX), Leflunomide (LEF), IV Methylprednisolone (MP) and No treatment (No Tx).

Mentions: A deep punch biopsy of the initial lesion demonstrated histologic findings consistent with LS (morphea), including full thickness dermal fibrosis with a superficial and deep perivascular lymphohistiocytic infiltrate. In addition, there was thickening of the fibrous septa in the panniculus with aggregates of plasma cells at the edges, supporting deeper involvement. An eosinophilic infiltrate was not present in the subcutis. She was initially treated with topical corticosteroids, but these were discontinued due to their ineffectiveness to halt disease with expansion of lesions noted. Oral treatment was initiated, though not considered as standard therapy, with penicillin VK and hydroxychloroquine (400mg daily) (Fig. 1). Approximately 1 year later she was started on more traditional steroid and methotrexate therapy, initially with oral prednisone, followed by IV methylprednisolone administered per Toronto protocol (1000 mg IV daily three consecutive days) [6]. Due to intolerability of the methotrexate (maximal dose 17.5 mg subcutaneous weekly), she was switched to mycophenolate mofetil (MMF) and continued on it for another year at a maximum dose of 1500 mg twice daily. She started to complain of odd side effects with MMF, including migraine headache and short term memory loss, leading to its eventual discontinuation. She received another course of monthly three consecutive days of 1000 mg daily IV methylprednisolone for 3 months prior to our evaluation in clinic (Fig. 1).


A Case Report of Successful Treatment of Recalcitrant Childhood Localized Scleroderma with Infliximab and Leflunomide.

Ferguson ID, Weiser P, Torok KS - Open Rheumatol J (2015)

Timeline of medical therapy. A graphic summary of the past 5 years of treatment for the patient reported in the case. Topicalcorticosteroids (Top CS), Penicillin VK (Pen VK), Hydroxychloroquine (HCQ), Prednisone (Pred), Methotrexate (MTX), Minocycline(MNC), Mycophenolate mofetil (MMF), Infliximab (IFX), Leflunomide (LEF), IV Methylprednisolone (MP) and No treatment (No Tx).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4493649&req=5

Figure 1: Timeline of medical therapy. A graphic summary of the past 5 years of treatment for the patient reported in the case. Topicalcorticosteroids (Top CS), Penicillin VK (Pen VK), Hydroxychloroquine (HCQ), Prednisone (Pred), Methotrexate (MTX), Minocycline(MNC), Mycophenolate mofetil (MMF), Infliximab (IFX), Leflunomide (LEF), IV Methylprednisolone (MP) and No treatment (No Tx).
Mentions: A deep punch biopsy of the initial lesion demonstrated histologic findings consistent with LS (morphea), including full thickness dermal fibrosis with a superficial and deep perivascular lymphohistiocytic infiltrate. In addition, there was thickening of the fibrous septa in the panniculus with aggregates of plasma cells at the edges, supporting deeper involvement. An eosinophilic infiltrate was not present in the subcutis. She was initially treated with topical corticosteroids, but these were discontinued due to their ineffectiveness to halt disease with expansion of lesions noted. Oral treatment was initiated, though not considered as standard therapy, with penicillin VK and hydroxychloroquine (400mg daily) (Fig. 1). Approximately 1 year later she was started on more traditional steroid and methotrexate therapy, initially with oral prednisone, followed by IV methylprednisolone administered per Toronto protocol (1000 mg IV daily three consecutive days) [6]. Due to intolerability of the methotrexate (maximal dose 17.5 mg subcutaneous weekly), she was switched to mycophenolate mofetil (MMF) and continued on it for another year at a maximum dose of 1500 mg twice daily. She started to complain of odd side effects with MMF, including migraine headache and short term memory loss, leading to its eventual discontinuation. She received another course of monthly three consecutive days of 1000 mg daily IV methylprednisolone for 3 months prior to our evaluation in clinic (Fig. 1).

Bottom Line: Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide.The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global assessments.Larger longitudinal studies or case series are needed to confirm and further investigate infliximab's role in localized scleroderma.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Division of Rheumatology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.

ABSTRACT
Herein we report successful treatment of an adolescent Caucasian female with severe progressive localized scleroderma (mixed subtype, including generalized morphea and linear scleroderma of the trunk/limb) using infliximab and leflunomide. The patient demonstrated improvement after the first 9 months of therapy based on her clinical examination, objective measures, and patient and parent global assessments. Infliximab is a potential treatment option for pediatric localized scleroderma patients who have progression of disease or who are unable to tolerate the side effect profile of more standard systemic therapy. Larger longitudinal studies or case series are needed to confirm and further investigate infliximab's role in localized scleroderma.

No MeSH data available.


Related in: MedlinePlus