Clinical characteristics and early recognition of thrombotic microangiopathy in children with systemic lupus erythematosus / 中国小儿急救医学

Objective:To analyze the clinical characteristics of children diagnosed with systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA)for early recognition.Methods:We retrospectively reviewed the clinical records of 14 SLE patients with TMA hospitalized at Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine from December 2005 to October 2020.Results:The incidence of TMA was 5.65%(14/248)of the hospitalized patients with SLE and 7.87%(14/178)of the hospitalized patients with lupus nephritis.Four patients were boys while ten patients were girls.One boy was six years old and other 13 patients were from 11 to 18 years old.Their SLEDAI scores ranged from 14 to 31, and all of them were severe activity.Renal biopsy of 11 patients during TMA course all revealed lupus nephritis(type Ⅳ, n=8; type Ⅳ+ Ⅴ, n=3). These 14 SLE children were diagnosed with TMA within 3 days to 2 months after admission.At the beginning of the hospitalization, only six patients had both anemia and thrombocytopenia, while eight patients only had moderate anemia.All of the patients had obvious hypocomplementemia.Especially in the patients with first onset of SLE without treatment, their serum levels of C3 were less than 0.17 g/L and C4 were less than 0.07 g/L.Moreover, glomerular filtration rates of these patients were lower than that in normal range.The follow-up time were 0.2-11.3 years(median time was 2.6 years). After treatment, six patients obtained complete remission, and five patients obtained partial remission.One patient had sudden death during the 4th plasmapheresis, and the other two patients deteriorated. Conclusion:Children with SLE and TMA are mostly in severe disease activity, and renal pathology is type Ⅳ lupus nephritis.The SLE children with anemia should be paid special attention to the level of serum complement whether they have thrombocytopenia or not.If the level of serum complements decrease obviously, glomerular filtration rates should be monitored closely and schistocytes should be searched repeatedly in the blood smears of the peripheral blood to facilitate the early recognition of TMA.

The clinical manifestations and thrombotic risk factors in primary antiphospholipid syndrome / 中华内科杂志

Objective To investigate the clinical characteristics in patients with primary antiphospholipid syndrome (PAPS) and to identify potential predictors of thrombotic events.Methods A total of 107 patients with PAPS were enrolled in our study, who were admitted in Peking Union Medical College Hospital from January 2004 to December 2014.Demographic data, age at onset, disease duration, past history of hypertension and regular cigarette smoking, clinical manifestations, imaging characteristics, management and prognosis were retrospectively collected.Bivariate statistical analysis and logistical regression test were performed to compare the discrepancy between patients with or without thromboembolic events.Results In 107 patients, there were 65 female and 42 male patients, with mean age (39.8 ± 15.8) years old, median disease duration 10.5 (2.0, 48.0) months.A total of 72(67.3％) patients reported episodes of thromboembolic events, including 72 venous thromboses and 29 arterial thromboses.The most frequent venous thromboses were deep vein thromboses (35.5％), pulmonary embolism the second common (29.9％), with cranial venous sinus thromboses the following (8.4％).In arterial thromboembolic events, the incidence of transient ischemic attack (TIA) and ischemic stoke was the highest (14.0％), embolism of lower extremities the second (6.5％) ,and 4 patients (3.7％) with acute myocardial infarction.Sixty seven patients (62.6％)had positive lupus anticoagulant, 60 patients (56.1％)with positive anticardiolipin antibody,32 patients (29.9％, 32/74) with positive β2 glycoprotein Ⅰ (β2GP I).Forty patients(37.4％)had double positive antibodies, while 19 cases (17.8％)with triple positive.In logistical regression, aging (per 10 years) and hypocomplementemia were significantly related to venous thrombosis (OR =1.421, 95％ CI 1.066-1.894, P ＜ 0.05, and OR =6.435, 95％ CI 1.374-30.130, P ＜ 0.05, respectively).Cigarette smoking and triple positive antibodies were independent risk factors of arterial thrombosis (OR =3.996, 95％ CI 1.079-14.795, P ＜ 0.05 and OR =3.166, 95％ CI 1.102-9.097, P ＜ 0.05, respectively).Conclusion Alas is an autoimmune disorder characterized by recurrent arterial and venous thromboembolic events.Venous thromboembolism is more common than the arterial.Age and hypocomplementemia are predictors of venous thromboembolism;while smoking and triple positive antibodies are independent risk factors of arterial thromboembolism.

A Case of Immunoglobulin G4-related Interstitial Nephritis with Bicytopenia

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease found in many organs including biliary tract, salivary gland, kidney, and lung. Tubulointerstitial nephritis is the most common renal manifestation, but hematologic involvement of IgG4-RD is rare. Here, we report on a case of a 57-year-old male with IgG4-related interstitial nephritis with bicytopenia, which was initially thought to be systemic lupus erythematosus. He presented with proteinuria, anemia, thrombocytopenia, and low complement levels. Histological findings showed an increased number of IgG4-positive plasma cells (>200/high power field), and an elevated IgG4/IgG ratio (>90%). Serum levels of IgG and IgG4 were also increased. This case emphasized the importance of differential diagnosis of IgG4-RD and immune complex glomerulonephritis.

Extraglandular Manifestations and Autoantibodies of Korean Patients with Primary Sjogren's Syndrome / 대한류마티스학회지

OBJECTIVE: To investigate the extraglandular manifestations and serological features of Korean patients with primary Sjogren's syndrome (pSS). METHODS: Clinical and laboratory data of 125 patients with pSS attending rheumatology clinic of Kangnam St. Mary's hospital, with a mean follow-up period of 64.8 months, were assessed retrospectively. RESULTS: Arthralgia was the most common extraglandular manifestation of pSS. Other extraglandular manifestations, such as myalgia, peripheral neuropathy, Raynaud's phenomenon, and hypothyroidism were frequently presented, but lymphoma was rare manifestation in Korean patients with pSS. Some extraglandular manifestations of pSS were associated with hypocomplementemia and antibodies against Ro, and La, and antinuclear antibody. CONCLUSION: The clinical manifestations and extraglandular manifestations of Korean patients with pSS patients were similar to other studies except low prevalence of lymphoma. Positivity of anti-Ro/SS-A, anti-La/SS-B antibodies and hypocomplementemia were closely associated with extraglandular manifestations in patients with pSS.

A Case of Focal Segmental Membranoproliferative Glomerulonephritis in a 5 Years Old Girl

=Abstract= Membranoproliferative glomerulonephritis (MPGN) is a progressive primary glomerulonephritis characterized by mesangial proliferation with increased mesangial matrix, subendothelial immune deposits, mesangial interposition and a double contour feature of the glomerular basement membrane. The glomerular involvement in MPGN is usually diffuse; however, cases of focal or segmental MPGN have been reported by several authors. We report a case of focal segmental MPGN with prolonged hypocomplementemia for 3 years in a 5 years old girl.

Prognosis of Acute Poststreptococcal Glomerulonephritis (APSGN) in Children

PURPOSE: Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A beta-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. METHODS: Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A beta-hemolytic streptococcal infection were evaluated. RESULTS: Six(12.5%) patients showed elevation of serum creatinine level but there was no patient with persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9%). Hematuria disappeared within 6 months(79%) and proteinuria within 6 months(100%) from the disease onset. CONCLUSION: Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4%) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequela.