AI Article Synopsis
- Systemic lupus erythematosus (SLE) is a complex autoimmune disease that can cause various organ issues, including autoimmune peripheral cytopenia, which can lead to a rare condition known as autoimmune myelofibrosis (AIMF).
- A case was reported involving a 34-year-old woman with AIMF, who exhibited symptoms like gingival bleeding, joint pain, and swelling, along with lab findings indicative of SLE.
- There is limited research on the link between AIMF and SLE; however, recognizing this connection is important in case of uncontrolled cytopenia in SLE patients, and treatment remains aligned with standard SLE management.
Article Abstract
Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, characterised by multi-organ affections. Haematological involvement is a common manifestation of SLE, consisting of autoimmune peripheral cytopenia. Autoimmune myelofibrosis (AIMF) is a rare cause of cytopenia in SLE; it could precede or be concurrent with the diagnosis of SLE. There are few studies that describe this association.
Case Description: We report a case of AIMF revealing the diagnosis of SLE in 34-year-old female, presented with episodes of gingival bleeding associated with peripheral inflammatory polyarthralgia, photosensitivity and deterioration of general condition. Clinical examination revealed a soft pitting oedema in the lower limbs. Laboratory investigations showed a pancytopenia, inflammatory biological syndrome, with positive 24-hour proteinuria and anti-native DNA antibodies. A bone marrow biopsy showed diffuse myelofibrosis associated with maturation disorders and no tumour infiltrate. Renal biopsy revealed proliferative glomerulonephritis class III with immune deposits.
Conclusion: The association of AIMF with SLE has been rarely reported, and it could be another cause for cytopenia in SLE.
Learning Points: Autoimmune myelofibrosis can be associated with systemic lupus erythematosus (SLE), even though it is rare.This association should be considered when pancytopenia is not well controlled during SLE, prompting a bone marrow biopsy to confirm the diagnosis.The therapeutic management of this association is the same as that used in SLE.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11152231 | PMC |
| http://dx.doi.org/10.12890/2024_004511 | DOI Listing |
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