Abstract
This article presents a case of fulminant interstitial lung disease (ILD) with macrophage activation syndrome (MAS) as a rare complication of adult-onset Still’s disease in a 43-year-old male patient. Initial presentation showed severe ILD. Early treatment focused on high-dose corticosteroids; however, disease remission could not be achieved. After an additional inflammatory focus and underlying malignancy were excluded, the triad of pancytopenia, fever, and high ferritin levels indicated MAS; bone marrow biopsy confirmed secondary hemophagocytic histiocytosis. Treatment with tocilizumab induced fast and effective therapeutic success.
Zusammenfassung
Es wird über den Fall einer schweren interstitiellen Pneumonie (ILD) mit Makrophagenaktivierungssyndrom (MAS) als seltene Komplikation eines Morbus Still des Erwachsenen berichtet. Das Krankheitsbild der 43-jährigen Patientin ähnelte zunächst einem schweren Schub einer ILD. Eine umfassende Hochdosis-Glukokortikoidgabe i.v. blieb ohne Erfolg. Nach Ausschluss eines sekundären Infektgeschehens und einer Neoplasie ergab letztlich die Trias aus Panzytopenie, hohem Fieber sowie steigenden Serum-Ferritin-Spiegeln den Verdacht eines MAS, welches via Knochenmarkbiopsie mit einer hämophagozytischen Histiozytose bestätigt wurde. Die Behandlung mit Tocilizumab brachte einen raschen und durchschlagenden Therapieerfolg.
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G. Zhang, W. Liu, X. Zhang, and L. Liu declare that they have no competing interests.
For this article no studies with human participants or animals were performed by any of the authors. All studies performed were in accordance with the ethical standards indicated in each case. Additional written informed consent was obtained from all individual participants or their legal representatives for whom identifying information is included in this article.
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M. O. Becker, Zürich
P. Hoff, Berlin
A. J. Hueber, Bamberg
F. Moosig, Neumünster
Guangfeng Zhang and Wenxue Liu contributed equally to this article as co-first authors. Xiao Zhang and Liang Liu contributed equally to this article as co-corresponding authors.
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Zhang, G., Liu, W., Zhang, X. et al. Rescued from desperation: adult-onset Still’s disease with life-threatening interstitial lung disease and macrophage activation. Z Rheumatol 81, 335–338 (2022). https://doi.org/10.1007/s00393-021-01063-7
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DOI: https://doi.org/10.1007/s00393-021-01063-7
Keywords
- Tocilizumab
- Adult-onset Still’s disease
- Interstitial lung disease
- Macrophage activation syndrome
- Treatment resistance