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Erschienen in: Wiener klinische Wochenschrift 19-20/2021

25.05.2020 | case report

Low dose intravenous immunoglobulin in addition to cyclophosphamide in systemic sclerosis

Single centre experience

verfasst von: MD, PhD Assistant professor Dijana Perković, MD Marin Petrić, MD Ivona Božić, MD Katarina Borić, MD, PhD Assistant professor Daniela Marasović Krstulović, MD, PhD Assistant professor Mislav Radić, MD, PhD Professor Dušanka Martinović Kaliterna

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 19-20/2021

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Summary

Aim

Systemic sclerosis (SSc) is a rare chronic disease characterized by pathologic collagen deposits in the skin and internal organs. Although it is considered to be an autoimmune disease, immunosuppressants have a limited effect on severe SSc. Intravenous immunoglobulins (IVIG) have shown favorable effects in patients with SSc by suppressing the action of profibrotic cytokines, so they could have additional effect on standard treatment such as cyclophosphamide (CYC). This article presents the immunomodulatory effect of low-dose IVIG in addition to CYC in the treatment of severe SSc in this center during the last 9 years.

Methods

This retrospective observational study analyzed the medical documentation of nine patients with SSc treated with low-dose IVIG (0.4 g/kg and month) together with intravenous CYC (600 mg/m2 and month). The therapeutic effect on lung and skin manifestations was assessed.

Results

Of the patients one had interstitial lung diseases (ILD), two had progressive skin diseases, and six had a combination of skin and lung involvement. The best results were achieved in skin changes, where complete healing of digital ulcers (DU) was recorded in every reported case. A decrease in the modified Rodnan skin score (mRSS) was noted in three patients and increased diffusion capacity of the lungs for carbon monoxide in another three patients.

Conclusion

The results of the study suggest that IVIG may be an additional treatment option together with CYC for patients for whom other therapies have failed, but further studies on the exact role of IVIG in the treatment of severe SSc are required.
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Metadaten
Titel
Low dose intravenous immunoglobulin in addition to cyclophosphamide in systemic sclerosis
Single centre experience
verfasst von
MD, PhD Assistant professor Dijana Perković
MD Marin Petrić
MD Ivona Božić
MD Katarina Borić
MD, PhD Assistant professor Daniela Marasović Krstulović
MD, PhD Assistant professor Mislav Radić
MD, PhD Professor Dušanka Martinović Kaliterna
Publikationsdatum
25.05.2020
Verlag
Springer Vienna
Erschienen in
Wiener klinische Wochenschrift / Ausgabe 19-20/2021
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI
https://doi.org/10.1007/s00508-020-01669-y

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