Clinical characteristics and outcome of IgG4-related disease with hypocomplementemia: a prospective cohort study

Background Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic, immune-mediated, and fibro-inflammatory disease. Hypocomplementemia was found in part of IgG4-RD patients especially in the setting of active disease. Objectives This study aimed to clarify the clinical features, treatment efficacy, and outcome in IgG4-RD patients with hypocomplementemia. Methods 312 IgG4-RD patients were recruited in our prospective cohort conducted in Peking Union Medical College Hospital. Patients were divided into hypocomplementemia group and normal complement group according to serum C3 and C4 levels measured at baseline before treatment. Low serum C3 levels (< 0.73 g/L) and/or C4 levels (< 0.10 g/L) were defined as hypocomplementemia. Demographic data, clinical characteristics, laboratory parameters, treatment, and outcome of two groups were analyzed and compared. Results Hypocomplementemia was identified in 65 (20.8%) cases of untreated IgG4-RD patients at baseline. The average age of hypocomplementemia group was 55.85 ± 10.89 years, with male predominance (72.3%). Compared with normal complement group, patients with hypocomplementemia were likely to have more involved organs, higher IgG4-RD responder index (IgG4-RD RI), and higher laboratory parameters such as counts of eosinophils, inflammatory markers, immunoglobulin G (IgG), IgG1, IgG3, IgG4, and IgE. In addition, lymph nodes, lacrimal gland, submandibular gland, parotid gland, paranasal sinus, bile ducts, and prostate gland were more commonly affected (p < 0.05). Serum C3 and C4 showed a significant positively correlation with each other. Both C3 and C4 were negatively correlated with the number of involved organs, IgG, IgG3, IgG4, and IgG4-RD RI, as well as positively correlated with IgA and hypersensitive C reactive protein (hsCRP). 64 (98.5%) patients responded quickly to initial therapy at a 3-month follow-up. Fifteen (23.1%) patients relapsed during follow-up with mean recurrence time of 14.2 ± 13.8 months. Compared with normal complement group, there was no significant difference of relapse rate in two groups (P = 0.401). Conclusions Clinical characteristics of IgG4-related disease with hypocomplementemia differ from normal complement group. Serum C3 and C4 at baseline before treatment could be biological markers for disease activity. IgG4-RD with hypocomplementemia responded well to treatment and had no significant difference of relapse rate in IgG4-RD with normal complement.

Systemic lupus erythematosus multiorgan flare with quiescent serologic markers

Systemic lupus erythematosus (SLE) can affect almost every organ with differing degrees of severity. Typically, SLE activity is associated with hypocomplimentaemia and elevated double-stranded DNA (dsDNA) levels. We describe a case of a severe multiorgan lupus flare including lupus cerebritis, autoimmune haemolytic anaemia, lupus nephritis and lupus myopericarditis with normal complement and dsDNA levels. This highlights the importance of understanding the heterogeneous nature of SLE flares.

Clinical characteristics and outcome of IgG4-related disease with hypocomplementemia: a prospective cohort study

Background Immunoglobulin G4 related disease (IgG4-RD) is a newly recognized systemic, immune-mediated and fibro-inflammatory disease. Hypocomplementemia was found in part of IgG4-RD patients especially in the setting of active disease. Objectives This study aimed to clarify the clinical features, treatment efficacy and outcome in IgG4-RD patients with hypocomplementemia. Methods 312 IgG4-RD patients were recruited in our prospective cohort conducted in Peking union medical college hospital. Patient’s demographic data, clinical characteristics, laboratory parameters, treatment and outcome were analyzed. Results Hypocomplementemia was identified in 65(20.8%) cases of untreated IgG4-RD patients at baseline. The average age of hypocomplementemia group was 55.85±10.89 years, with male predominance (72.3%). Compared with normal complement group, patients with hypocomplementemia were likely to have more involved organs,higher IgG4-RD responder index (IgG4-RD RI), higher laboratory parameters such as counts of eosinophils, inflammatory markers༌immunoglobulin G(IgG), IgG1, IgG3, IgG4 and IgE. In addition, lymph nodes, lacrimal gland༌submandibular gland༌parotid gland༌paranasal sinus༌bile ducts and prostate gland were more commonly affected(p < 0.05). Serum C3 and C4 were negatively correlated with the number of involved organs, IgG4-RD RI, hypersensitive C-reactive protein (hsCRP), IgG, IgG1, IgG3 and IgG4. 64(98.5%) patients responded quickly to initial therapy at 3-month follow-up. Fifteen (23.1%) patients relapsed during follow-up with mean recurrence time of 14.2±13.8 months. Compared with normal complement group, there was no significant difference of relapse rate in two groups (P = 0.7559). Conclusions Clinical characteristics of IgG4-related disease with hypocomplementemia differs from normal complement group.Serum C3 and C4 at baseline before treatment could be biological markers for disease activity. IgG4-RD with hypocomplementemia responded well to treatment and had no significant difference of relapse rate in IgG4-RD with normal complement.

Groups whose lattices of normal subgroups are factorial

We prove that the groups G for which the lattice of normal subgroups N(G) is factorial are exactly the UND-groups, that is the groups for which every normal subgroup have a unique normal complement, with finite length.

Changing sex for selfish gain: B chromosomes of Lake Malawi cichlid fish

B chromosomes are extra, non-essential chromosomes present in addition to the normal complement of A chromosomes. Many species of cichlid fish in Lake Malawi carry a haploid, female-restricted B chromosome. Here we show that this B chromosome exhibits drive, with an average transmission rate of 70%. The offspring of B-transmitting females exhibit a strongly female-biased sex ratio. Genotyping of these offspring reveals the B chromosome carries a female sex determiner that is epistatically dominant to an XY system on linkage group 7. We suggest that this sex determiner evolved to enhance the meiotic drive of the B chromosome. This is some of the first evidence that female meiotic drive can lead to the invasion of new sex chromosomes solely to benefit the driver, and not to compensate for skewed sex ratios.