Refractory lupus nephropathy and acquired Factor <scp>VIII</scp> and <scp>IX</scp> deficiencies in a patient with systemic lupus erythematosus treated with rituximab

Cui, Qingya; Wu, Tianqin; Shen, H.-s.; Chen, H.-f.; Yu, Ziqiang; Wang, Z.-y.

doi:10.1111/hae.13232

Cited by 1 publication

(2 citation statements)

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“…No relapse during the 5-year follow-up with chloroquine 250 mg/ day, CsA 200 mg/day and mPSN in a maintenance dose. Cui et al 8 42y/M Asia Gradually enlarged subcutaneous hematoma on the right scapula, sporadic skin ecchymosis, and painful swelling for 2 months APTT 121.9s; FVIII activity 1%; FIX activity 4%; FVIII and FIX inhibitors levels were 30 and 15 BU; Proteinuria was 4.5 g per 24 hours; ANA 1:160+, anti-dsDNA 1:160+, anti-SSA+, anti-SSB+ SLE Failed to respond to aPCC and PED 60 mg/day for 6 weeks. Weekly Rit at 375 mg/m2 for four consecutive weeks Achieved partially remission with no haemorrhagic manifestations during the 1-year follow-up.…”

Section: Systematic Reviewmentioning

confidence: 99%

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Acquired Hemophilia Associated with Rheumatic Diseases: A Case-Based Systematic Review

Qi¹,

Liao²,

Xie³

2022

JIR

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To strengthen the understanding of rheumatic diseases (RDs) as the most common underlying conditions associated with acquired hemophilia (AH), a potentially fatal bleeding condition due to the development of autoantibodies or inhibitors to coagulation factor VIII, and rarely to factor IX, here we presented two cases of RDs associated AH to elucidate the disease progression, treatment, and prognosis. The presented 2 cases showed good responses to glucocorticoid (GC) and immunosuppressive agents. And then, a case-based systematic review was conducted to better understand the clinically practiced diagnosis and treatment of RDs associated AH. A total of 14 articles were included in the final literature review. All the identified 14 patients with underlying RDs and AH presented with bleeding symptoms, increased APTT, decreased FVIII activity, and positive FVIII inhibitors. Twelve of the 14 patients (85.7%) started an eradication of autoantibodies treatment with GC and immunosuppressive agents. Among which six patients achieved partial or complete remission, and four patients (28.6%) switched to Rituximab and responded well. Nine of the 14 patients received hemostasis therapy, including recombinant human FVIIa (rFVIIa). Two patients (14.3%) died due to mass bleeding and key organ failure. AH should be highly suspected in patients with RDs presenting spontaneous mucocutaneous or internal bleeding and an isolated prolonged APTT. Given the high morbidity of AH, it is important to facilitate efficient and proper management.

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Section: Systematic Reviewmentioning

confidence: 99%

“…After excluding reviews, duplicates, and articles with too little clinical data, a total of 14 articles were included in the final literature review. [3][4][5][6][7][8][9][10][11][12][13][14][15][16] Detailed characteristics were listed in Table 1. SLE (7/14) and rheumatoid arthritis (RA) (4/14) were the most common concurrent RDs.…”

Section: Systematic Reviewmentioning

confidence: 99%