Blood-dominant disease in late-and-early-onset lupus: A systematic review and meta-analysis.

OBJECTIVES: Numerous studies have explored hematological manifestations in early-onset systemic lupus erythematosus (erSLE) (age ≤ 50) and late-onset SLE (ltSLE) patients (age > 50), yielding diverse results. This study employs a meta-analysis to examine differences in hematologic manifestations between ltSLE and erSLE. METHODS: Studies investigating the frequency of hematological manifestations in ltSLE patients were included. The frequencies of autoimmune hemolytic anemia (AIHA), thrombocytopenia (TP), lymphopenia, leukopenia, lymphadenopathy, and thrombosis were compared between erSLE and ltSLE groups. Two authors independently reviewed and assessed data consistency among abstracts, tables, and text to mitigate bias. Forest plots were utilized to compare odds ratios (95 % CI) of hematological manifestations by age groups, and study heterogeneity was evaluated using I2. RESULTS: The analysis included 39 eligible studies with 19,103 SLE patients (16,314 erSLE, 2789 ltSLE). Among these studies, 28 reported AIHA which was found to be more frequent in erSLE (OR = 1.29, 95 %CI = 1.11-1.39, p = 0.0008). Twenty studies provided data on lymphopenia which was found to be more frequent in erSLE (OR = 1.184, 95 %CI = 1.063-1.318, p = 0.0021). 32 studies included data on leukopenia and the frequency was higher in erSLE (OR: 1.338, 95 %CI: 1.22-1.47, p < 0.0001). Lymphadenopathy was more prevalent in erSLE (OR = 2.32, 95 % CI = 1.61-3.34, p < 0.0001). No significant difference was observed in thrombosis and TP frequency between the two groups. CONCLUSION: Attributing hematological findings to SLE in late-onset patients presents challenges due to comorbidities and polypharmacy. Overall, the frequencies of AIHA, lymphopenia, leukopenia, and lymphadenopathy were more common in erSLE patients compared to ltSLE in this study.

Anti-PL-12 anti-synthetase syndrome manifesting with multiple digital ischemia: Case report & review of the literature.

Key Clinical Message: Acute digital ischemia is a rare manifestation of anti-synthetase syndrome in the absence of Raynaud's phenomenon. A high index of suspicion may result in early diagnosis and better clinical outcomes. Abstract: A 61-year-old male patient was admitted to the hospital for worsening arthralgias with morning stiffness lasting hours, as well as left sided headaches, and jaw pain while eating. He had significant weight loss and subjective fever at home. Multiple fingers and toes were noted to be ischemic. His laboratory workup was pertinent for significantly elevated inflammatory markers, and mild Creatinine kinase elevation. Chest imaging and later lung biopsy were notable for organizing pneumonia. Conventional angiogram showed evidence of significant digital disease without collaterals. Subsequent autoimmune screening tests with extended myositis-specific and myositis-associated panels revealed a strongly positive anti-PL-12 antibody and moderately positive anti- SSA-52KD IgG ab. After ruling out infectious etiologies and malignancy, anti-synthetase syndrome (ASyS) diagnosis was considered in the presence of ischemic digits, organizing pneumonia, polyarthralgia, constitutional symptoms, increased inflammatory markers and positive antibodies. The patient was treated with high dose prednisone and mycophenolate mofetil along with amlodipine and sildenafil for digital vasodilation. Acute digital ischemia may be the first manifestation of ASyS with ILD. A high index of suspicion is warranted for early diagnosis and better outcomes.