An overlapping case of IgG4-related disease and systemic lupus erythematosus treated with belimumab : a case-based review
© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature..
IgG4-related disease (IgG4-RD) is a systemic condition in which IgG4+ plasma cell infiltration and fibrosis cause organ swelling and lead to diverse clinical manifestations. Although IgG4-RD typically responds to glucocorticoids (GCs), relapse during tapering occurs and an early GC-sparing approach might therefore be beneficial. Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with multiple symptoms that is also treated with GCs as a first-line therapy. Recently, belimumab, a recombinant human IgG-1λ monoclonal antibody that inhibits B-cell activating factor, was approved, but reports of use for IgG4-RD are scarce. Here, we present a rare case of IgG4-RD complicated with SLE which was successfully treated with belimumab. A 67-year-old man was diagnosed with IgG4-RD based on a high serum IgG4 level and histopathological findings. Furthermore, he had pericardial effusion on echocardiography, and laboratory tests revealed thrombocytopenia, autoimmune hemolysis, positive anti-nuclear antibodies, positive anti-DNA antibodies, and hypocomplementemia. These data led to an SLE diagnosis. Treatment was started with prednisolone at 40 mg/day, plus hydroxychloroquine, which initially improved both the SLE and IgG4-RD symptoms. During the GC tapering, belimumab was added and clinical symptoms resolved completely. Our case and the literature review summarize reported rare overlapping cases of IgG4-RD and SLE and suggest that belimumab is a promising candidate for the treatment of IgG4-RD.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2024 |
|---|---|
| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:44 |
|---|---|
| Enthalten in: |
Rheumatology international - 44(2024), 3 vom: 27. Feb., Seite 549-556 |
| Sprache: |
Englisch |
|---|
| Beteiligte Personen: |
Iwamoto, Megumi [VerfasserIn] |
|---|
| Links: |
|---|
| Anmerkungen: |
Date Completed 16.02.2024 Date Revised 16.02.2024 published: Print-Electronic Citation Status MEDLINE |
|---|
| doi: |
10.1007/s00296-023-05510-3 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM366608339 |
|---|
| LEADER | 01000caa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM366608339 | ||
| 003 | DE-627 | ||
| 005 | 20240216232717.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 240108s2024 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1007/s00296-023-05510-3 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1295.xml |
| 035 | |a (DE-627)NLM366608339 | ||
| 035 | |a (NLM)38170205 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Iwamoto, Megumi |e verfasserin |4 aut | |
| 245 | 1 | 3 | |a An overlapping case of IgG4-related disease and systemic lupus erythematosus treated with belimumab |b a case-based review |
| 264 | 1 | |c 2024 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 16.02.2024 | ||
| 500 | |a Date Revised 16.02.2024 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. | ||
| 520 | |a IgG4-related disease (IgG4-RD) is a systemic condition in which IgG4+ plasma cell infiltration and fibrosis cause organ swelling and lead to diverse clinical manifestations. Although IgG4-RD typically responds to glucocorticoids (GCs), relapse during tapering occurs and an early GC-sparing approach might therefore be beneficial. Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with multiple symptoms that is also treated with GCs as a first-line therapy. Recently, belimumab, a recombinant human IgG-1λ monoclonal antibody that inhibits B-cell activating factor, was approved, but reports of use for IgG4-RD are scarce. Here, we present a rare case of IgG4-RD complicated with SLE which was successfully treated with belimumab. A 67-year-old man was diagnosed with IgG4-RD based on a high serum IgG4 level and histopathological findings. Furthermore, he had pericardial effusion on echocardiography, and laboratory tests revealed thrombocytopenia, autoimmune hemolysis, positive anti-nuclear antibodies, positive anti-DNA antibodies, and hypocomplementemia. These data led to an SLE diagnosis. Treatment was started with prednisolone at 40 mg/day, plus hydroxychloroquine, which initially improved both the SLE and IgG4-RD symptoms. During the GC tapering, belimumab was added and clinical symptoms resolved completely. Our case and the literature review summarize reported rare overlapping cases of IgG4-RD and SLE and suggest that belimumab is a promising candidate for the treatment of IgG4-RD | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Review | |
| 650 | 4 | |a Belimumab | |
| 650 | 4 | |a IgG4-related disease | |
| 650 | 4 | |a Systemic lupus erythematosus | |
| 650 | 7 | |a belimumab |2 NLM | |
| 650 | 7 | |a 73B0K5S26A |2 NLM | |
| 650 | 7 | |a Antibodies, Monoclonal, Humanized |2 NLM | |
| 650 | 7 | |a Antibodies, Monoclonal |2 NLM | |
| 650 | 7 | |a Glucocorticoids |2 NLM | |
| 650 | 7 | |a Immunoglobulin G |2 NLM | |
| 650 | 7 | |a Immunosuppressive Agents |2 NLM | |
| 700 | 1 | |a Asashima, Hiromitsu |e verfasserin |4 aut | |
| 700 | 1 | |a Sugita, Toshiki |e verfasserin |4 aut | |
| 700 | 1 | |a Kawashima, Fumina |e verfasserin |4 aut | |
| 700 | 1 | |a Sugita, Naoki |e verfasserin |4 aut | |
| 700 | 1 | |a Rai, Akiyoshi |e verfasserin |4 aut | |
| 700 | 1 | |a Kuroda, Yuki |e verfasserin |4 aut | |
| 700 | 1 | |a Kawashima, Akira |e verfasserin |4 aut | |
| 700 | 1 | |a Tabuchi, Daiki |e verfasserin |4 aut | |
| 700 | 1 | |a Akao, Satoshi |e verfasserin |4 aut | |
| 700 | 1 | |a Sato, Ryota |e verfasserin |4 aut | |
| 700 | 1 | |a Nishiyama, Taihei |e verfasserin |4 aut | |
| 700 | 1 | |a Toko, Hirofumi |e verfasserin |4 aut | |
| 700 | 1 | |a Honda, Fumika |e verfasserin |4 aut | |
| 700 | 1 | |a Ohyama, Ayako |e verfasserin |4 aut | |
| 700 | 1 | |a Kitada, Ayako |e verfasserin |4 aut | |
| 700 | 1 | |a Abe, Saori |e verfasserin |4 aut | |
| 700 | 1 | |a Miki, Haruka |e verfasserin |4 aut | |
| 700 | 1 | |a Hagiwara, Shinya |e verfasserin |4 aut | |
| 700 | 1 | |a Kondo, Yuya |e verfasserin |4 aut | |
| 700 | 1 | |a Tsuboi, Hiroto |e verfasserin |4 aut | |
| 700 | 1 | |a Matsumoto, Isao |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Rheumatology international |d 1985 |g 44(2024), 3 vom: 27. Feb., Seite 549-556 |w (DE-627)NLM012644315 |x 1437-160X |7 nnns |
| 773 | 1 | 8 | |g volume:44 |g year:2024 |g number:3 |g day:27 |g month:02 |g pages:549-556 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1007/s00296-023-05510-3 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 44 |j 2024 |e 3 |b 27 |c 02 |h 549-556 | ||