Significance of the weighted complement domain of the EULAR/ACR classification criteria in juvenile onset systemic lupus erythematosus.

OBJECTIVE: We aimed to compare the clinical and laboratory characteristics of patients with SLE according to the weighted complement status of the EULAR/ACR criteria and investigate whether different weighting of the complement status at disease onset is associated with outcomes. METHODS: Patients diagnosed with juvenile onset SLE who fulfilled the 2019 EULAR/ACR classification criteria were retrospectively analyzed. RESULTS: Among 43 patients included, hypocomplementemia was observed in 37 (86%), mostly with a low level of both complement C3 (C3) and complement C4 (C4) (53.5%). In patients with low levels of both C3 and C4, more common cutaneous (65.2% vs 28.6%, p: 0.045), musculoskeletal involvement (78.3% vs 42.9%, p: 0.039), autoimmune hemolytic anemia (52.2% vs 14.3%, p: 0.035), positive anti-dsDNA (65.2% vs 21.4%, p: 0.017) and anti-Sm antibodies (60.9% vs 21.4%, p: 0.04) were observed. In addition these patients had higher scores from the 2019 EULAR/ACR classification criteria (26 vs 15.5, p: < 0.0001). Remission and flare rates, and SLE associated damage were not differed according to the complement status in patients with hypocomplementemia. CONCLUSION: Observation of more frequent clinical and serological activity with higher total scores from the EULAR/ACR classification criteria supported the higher scoring of patients with low C3 and C4 in the weighted criteria. However, since significant number of patients did not exhibit low complement C4, and the frequency of kidney involvement did not differ according to the weighted complement status, complement C3 might be suggested as a more important diagnostic tool in patients with juvenile onset SLE. Also, weighted complement status at onset did not seem to affect the disease outcomes.

Hematologic manifestations of juvenile systemic lupus erythematosus: An emphasis on anemia.

OBJECTIVE: Anemia is common in patients with juvenile systemic lupus erythematosus (jSLE). While autoimmune hemolytic anemia (AIHA) is the only etiology included in the classification criteria, the etiology of anemia in jSLE may be diverse. We aimed to investigate the etiology of anemia in jSLE and the relationship between anemia and disease characteristics at onset and during the follow-up period. METHODS: Patients diagnosed with jSLE who met the Systemic Lupus Erythematosus International Collaborating Clinics classification criteria between January 2012 and December 2020 were retrospectively analyzed. RESULTS: Hematologic involvement was observed in 70% of the patients. Anemia was the most common cytopenia among patients (60%). Anemia of chronic disease (ACD) and AIHA were the most common etiological factors, both observed in 23% of patients. Patients with anemia had a significantly higher rate of positive ds-DNA antibody and higher erythrocyte sedimentation rate (ESH) and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) scores. ESH, serum ferritin, and SLEDAI scores negatively correlated with hemoglobin levels in patients with anemia. Iron deficiency was the sole etiology of new-onset anemia. Patients with new-onset anemia during the follow-up period had significantly lower hemoglobin values at onset and a higher rate of renal involvement. CONCLUSION: Anemia in jSLE is mostly AIHA and ACD, but iron deficiency is not rare. The severity of inflammation is associated with the severity of anemia. During the follow-up period, iron deficiency was the predominant cause of anemia, especially in patients with lower hemoglobin concentrations at onset and renal involvement.

Cutaneous, systemic features and laboratory characteristics of late- versus adult-onset systemic lupus erythematosus in 1006 Thai patients.

BACKGROUND: Age at disease onset may modulate systemic lupus erythematosus (SLE), but its relation to cutaneous/extracutaneous manifestation remains understudied. OBJECTIVE: To compare the cutaneous, systemic features, laboratory characteristics, and disease severity between late- and adult-onset SLE patients. METHODS: Analyses of the cutaneous, systemic involvement, laboratory investigations, SLE disease activity index 2000 (SLEDAI-2K), and disease damage were performed to compare between groups. RESULTS: Of 1006 SLE patients, 740 and 226 had adult- (15-50 years) and late-onset (>50 years), respectively. Among 782 with cutaneous lupus erythematosus (CLE), acute CLE (ACLE) and chronic CLE (CCLE) were more common in the adult- and late-onset SLE, respectively (p = 0.001). Multivariable logistic regression analysis demonstrated that male patients and skin signs, including papulosquamous subacute CLE, discoid lupus erythematosus, and lupus profundus, were associated with late-onset SLE (all p < 0.05). Late-onset SLE had lower lupus-associated autoantibodies, and systemic involvement (all p < 0.05). ACLE, CCLE, mucosal lupus, alopecia, and non-specific lupus were related to higher disease activity in adult-onset SLE (all p < 0.001). There was no difference in the damage index between the two groups. CONCLUSIONS: Late-onset SLE had a distinct disease expression with male predominance, milder disease activity, and lower systemic involvement. Cutaneous manifestations may hold prognostic values for SLE.

A Case of Behcet's Disease Associated with Myelofibrosis

Behcet's disease (BD) is a systemic vasculitis of unknown etiology that is rarely observed in association with leukemia and other hematologic disorders. We recently encountered a patient who presented with BD associated with myelofibrosis that was not attributable to other underlying causes. The patient was a 49-year-old man with a 3-year history of probable BD diagnosed by clinical findings; he was admitted because of anemia and splenomegaly. Bone marrow examination revealed myelofibrosis. After steroid therapy, the patient's symptoms of BD improved, and his hemoglobin level recovered. Therefore, we believe that the patient had BD with bone marrow involvement.

Clinical features of newly-diagnosed primary Sj(o)gren's syndrome: analysis of 86 cases / 中华风湿病学杂志

Objective To assess the clinical features of newly diagnosed primary Sjogren's syndrome (pSS). Methods Patients were diagnosed according to the international consensus criteria for Sjogren's Syndrome published in 2002. Clinical manifestations and laboratory tests of 86 pSS cases hospitalized in Nanjing Drum Tower Hospital in the past two years were reviewed. Results Among the 86 patients, 95.3% were female and the average disease onset age was 38.6 years. The median time from disease onset to diagnosis was 6 months. Dry mouth, dry eyes and arthralgia were the most common symptoms. Hematologic involvement was found to be prominent in these patients (69.8%). The incidence of abnormal liver function, interstitial lung disease and pulmonary arterial hypertension was 19.8%, 8.2% and 5.8% simultaneously. Younger patients (less than 18 years old) had lower frequency of dry mouth and dry eyes but higher ffrequency of fever and lymph nodes enlargement than the elderly patients (P<0.05). Patients with positive anti-SSA or anti-SSB antibodies had higher incidence of hematological changes as well as ESR than those with negative auto-antibodia. Elevated globulin/IgG and positive antinuclear antibody or rheumatoid factor (P< 0.05). Conclusion pSS is not always a benign disease. Some patients will develop vital organ damages very early and thus need to be identified and treated in time. It should not be overemphasize the importance of dry mouth and dry eyes for the diagnosis of pSS, especially in young patients. Those patients who have fever, high globulin level and positive rheumatoid factor of unknown origin should be screened for pSS.