Evidence map›Paper›PMID 40956308›Full record

ReviewRheumatology international2025

Simultaneous onset of systemic sclerosis and light chain amyloidosis: the first report of a case report and review of the literature.

Anna Colangelo, Francesco Tromby, Elisabetta Agliani, Lorenza Bruno, Giacomo Cafaro, Federico Crusco, Anna Mengoni, Cinzia Zuchi, Roberto Gerli, Elena Bartoloni and 1 more

Abstract readCase ReportsReview
In one paragraph

Review in Rheumatology international, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

11 authors.

Anna Colangelo *Section of Rheumatology, Department of Medicine and Surgery, University of Perugia, Piazzale Giorgio Menghini, 1, Perugia, PG, 06129, Italy.ORCID http://orcid.org/0009-0000-1934-4557
Francesco Tromby *Section of Rheumatology, Department of Medicine and Surgery, University of Perugia, Piazzale Giorgio Menghini, 1, Perugia, PG, 06129, Italy.ORCID http://orcid.org/0009-0006-2805-9313
Elisabetta AglianiHematology and Bone Marrow Transplantation Unit, Department of Medicine and Surgery, University of Parma, Parma, Italy.
Lorenza BrunoSection of Rheumatology, Department of Medicine and Surgery, University of Perugia, Piazzale Giorgio Menghini, 1, Perugia, PG, 06129, Italy.ORCID http://orcid.org/0000-0002-2849-145X
Giacomo CafaroSection of Rheumatology, Department of Medicine and Surgery, University of Perugia, Piazzale Giorgio Menghini, 1, Perugia, PG, 06129, Italy.ORCID http://orcid.org/0000-0003-1774-1916
Federico CruscoUSL Umbria 2 "Nuovo Ospedale San Giovanni Battista", Radiologia, Foligno, Italy.
Anna MengoniCardiology and Cardiovascular Pathophysiology, S. Maria della Misericordia Hospital, University of Perugia, Perugia, Italy.ORCID http://orcid.org/0000-0003-2924-1409
Cinzia ZuchiCardiology and Cardiovascular Pathophysiology, S. Maria della Misericordia Hospital, University of Perugia, Perugia, Italy.ORCID http://orcid.org/0000-0003-3955-3879
Roberto GerliSection of Rheumatology, Department of Medicine and Surgery, University of Perugia, Piazzale Giorgio Menghini, 1, Perugia, PG, 06129, Italy.ORCID http://orcid.org/0000-0002-4684-575X
Elena Bartoloni *Section of Rheumatology, Department of Medicine and Surgery, University of Perugia, Piazzale Giorgio Menghini, 1, Perugia, PG, 06129, Italy.ORCID http://orcid.org/0000-0003-4776-2136
Carlo Perricone *Section of Rheumatology, Department of Medicine and Surgery, University of Perugia, Piazzale Giorgio Menghini, 1, Perugia, PG, 06129, Italy. carlo.perricone@unipg.it.ORCID http://orcid.org/0000-0003-4771-6981

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Systemic sclerosis (SSc) and amyloidosis are rare, complex conditions that impair the function of multiple organs, each with distinct pathogenic mechanisms: autoimmunity for SSc and misfolded protein deposition for amyloidosis. We present the first documented case of a 57-year-old woman with coexisting SSc and systemic AL amyloidosis with multi-organ involvement, in which treatment for amyloidosis led to a notable improvement in SSc symptoms. The patient presented experiencing fatigue, exertional dyspnea, epigastric pain and syncopal episodes in the summer of 2023. Investigations revealed mild increase in left ventricle thickness, elevated NT-proBNP and troponin with negative coronary angiography. She was subsequently diagnosed with SSc with multi-organ involvement and systemic AL amyloidosis confirmed by biopsy. Treatment with a modified Dara-CyBorD protocol led to improvement in SSc symptoms, especially in terms of dyspnea and skin involvement. This is the first reported case of SSc coexisting with systemic AL amyloidosis. The patient responded well to therapy for amyloidosis, suggesting potential overlapping treatment benefits. A multidisciplinary approach was essential, and further studies are needed to explore therapeutic interactions between these two rare diseases.

Indexed as

Immunoglobulin Light-chain AmyloidosisScleroderma, SystemicFemaleHumansMiddle AgedAmyloidosisanti-CD38DaratumumabLight-chain immunoglobulinSystemic sclerosis

Identifiers

PMID40956308
PMCPMC12441099

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.