# Two Cases of Systemic Lupus Erythematosus With Castleman Disease

**Authors:** Ana M Bravo-Andrade, Jaime A Ibarra-Burgos, Juliana M Bacca-Gonzalez, Jorge A Torres-Trujillo

PMC · DOI: 10.7759/cureus.102719 · Cureus · 2026-01-31

## TL;DR

This paper reports two rare cases where systemic lupus erythematosus co-occurred with Castleman disease, both successfully treated with rituximab.

## Contribution

The paper presents two new clinical cases of a rare association between systemic lupus erythematosus and plasma cell-subtype Castleman disease.

## Key findings

- Two Latin-American women with systemic lupus erythematosus had generalized lymphadenopathy and plasma cell-subtype Castleman disease.
- Both patients showed adequate response to rituximab treatment.
- The cases highlight diagnostic challenges due to overlapping clinical features.

## Abstract

Systemic lupus erythematosus is a heterogeneous autoimmune disease that commonly presents with lymphadenopathy, a feature that can lead to confusion with other diseases due to its non-specificity. Histopathological features are usually nonspecific and may mimic other conditions. Other cases have described Castleman disease, a rare lymphoproliferative disorder, in patients with systemic lupus erythematosus. This uncommon association poses diagnostic challenges due to the clinical overlap. We present cases of two Latin-American women with systemic lupus erythematosus and generalized lymphadenopathy, with histopathological findings compatible with plasma cell-subtype Castleman disease, who showed adequate response to rituximab treatment.

## Linked entities

- **Diseases:** Systemic lupus erythematosus (MONDO:0007915), Castleman disease (MONDO:0015564)

## Full-text entities

- **Genes:** SDC1 (syndecan 1) [NCBI Gene 6382] {aka CD138, SDC, SYND1, syndecan}, RNPC3 (RNA binding region (RNP1, RRM) containing 3) [NCBI Gene 55599] {aka CPHD7, IGHD5, RBM40, RNP, SNRNP65}, KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, APOH (apolipoprotein H) [NCBI Gene 350] {aka B2G1, B2GP1, BG}, HLA-G (major histocompatibility complex, class I, G) [NCBI Gene 3135] {aka MHC-G}, SSB (small RNA binding exonuclease protection factor La) [NCBI Gene 6741] {aka LARP3, La, La/SSB, SSB/La}, FCER2 (Fc epsilon receptor II) [NCBI Gene 2208] {aka BLAST-2, CD23, CD23A, CLEC4J, FCE2, FCErII}, LANA [NCBI Gene 4961527], CCND1 (cyclin D1) [NCBI Gene 595] {aka BCL1, D11S287E, PRAD1, U21B31}, CD5 (CD5 molecule) [NCBI Gene 921] {aka LEU1, T1}, SOX11 (SRY-box transcription factor 11) [NCBI Gene 6664] {aka CSS9, IDDMOH, MRD27}, TNFRSF8 (TNF receptor superfamily member 8) [NCBI Gene 943] {aka CD30, D1S166E, Ki-1}, IL6 (interleukin 6) [NCBI Gene 3569] {aka BSF-2, BSF2, CDF, HGF, HSF, IFN-beta-2}, MME (membrane metalloendopeptidase) [NCBI Gene 4311] {aka CALLA, CD10, CMT2T, NEP, SCA43, SFE}, CRP (C-reactive protein) [NCBI Gene 1401] {aka PTX1}, BCL2 (BCL2 apoptosis regulator) [NCBI Gene 596] {aka Bcl-2, PPP1R50}, MTOR (mechanistic target of rapamycin kinase) [NCBI Gene 2475] {aka FRAP, FRAP1, FRAP2, RAFT1, RAPT1, SKS}, BCL6 (BCL6 transcription repressor) [NCBI Gene 604] {aka BCL5, BCL6A, LAZ3, ZBTB27, ZNF51}, SPN (sialophorin) [NCBI Gene 6693] {aka CD43, GALGP, GPL115, LEU-22, LSN}
- **Diseases:** CMV (MESH:D003586), splenomegaly (MESH:D013163), dengue (MESH:D003715), autoimmune (MESH:D001327), leukopenia (MESH:D007970), Lymphadenopathy (MESH:D008206), myocarditis (MESH:D009205), chest pain (MESH:D002637), proteinuria (MESH:D011507), fever (MESH:D005334), retroperitoneal and pelvic adenopathies (MESH:C538370), IgG4 disease (MESH:D000077733), cervical and axillary lymphadenopathy (MESH:D002575), SLE (MESH:D008180), MCD (MESH:D012514), syphilis (MESH:D013587), EBV (MESH:D020031), inflammation (MESH:D007249), CD (MESH:D005871), hematuria (MESH:D006417), Sjogren's syndrome type B (MESH:D012859), lupus nephritis (MESH:D008181), malignancy (MESH:D009369), adenopathy (MESH:D000072281), hyaline vascular lesions (MESH:D014652), dyspnea (MESH:D004417), leptospirosis (MESH:D007922), hepatitis B and C (MESH:D006509), Thrombocytosis (MESH:D013922), lymphoproliferative disorder (MESH:D008232), lymphoma (MESH:D008223), oral candidiasis (MESH:D002180), palpitations (MESH:D006331), tuberculosis (MESH:D014376), polyclonal hypergammaglobulinemia (MESH:D006942), hemolytic anemia (MESH:D000743), necrosis (MESH:D009336), hyperplasia (MESH:D006965), autoimmune destruction (MESH:D008105), asthenia (MESH:D001247), malaria (MESH:D008288), immune dysregulation (OMIM:614878), HIV (MESH:D015658), toxoplasmosis (MESH:D014123), autoimmune, infectious or hematological disorders (MESH:D003141), chronic (MESH:D002908), autoimmune hemolytic anemia (MESH:D000744), anemia (MESH:D000740), neutrophilia (MESH:C563010), Hepatomegaly (MESH:D006529), viral infections (MESH:D014777), complement deficiencies (MESH:D007153), hematological compromise (MESH:D006402), thrombocytopenia (MESH:D013921), infections (MESH:D007239), plasmacytoma (MESH:D010954), leukocytosis (MESH:D007964), weight loss (MESH:D015431), arthralgia (MESH:D018771), antiphospholipid syndrome (MESH:D016736)
- **Chemicals:** methylprednisolone (MESH:D008775), cyclophosphamide (MESH:D003520), mycophenolate (MESH:D009173), azathioprine (MESH:D001379), steroid (MESH:D013256), prednisolone (MESH:D011239), hydroxychloroquine (MESH:D006886), Rituximab (MESH:D000069283)
- **Species:** Human gammaherpesvirus 8 (no rank) [taxon 37296], Staphylococcus aureus (species) [taxon 1280], human gammaherpesvirus 4 (Epstein Barr virus, no rank) [taxon 10376], Homo sapiens (human, species) [taxon 9606], Haemophilus influenzae (species) [taxon 727]

## Full text

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## Figures

6 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12950997/full.md

## References

21 references — full list in the complete paper: https://tomesphere.com/paper/PMC12950997/full.md

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Source: https://tomesphere.com/paper/PMC12950997