Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients

Abstract

BackgroundThe aim of this study was to assess the outcomes of remission induction in patients with IgG4-related disease (IgG4-RD) in our cohort, and to investigate the characteristics, prognosis, and risk factors in the patients failed of remission induction.MethodsWe prospectively enrolled 215 newly diagnosed patients with IgG4-RD, who were initially treated with glucocorticoid (GC) alone or in combination with immunosuppressive agents (IM), and had at least 6 months of follow up. The therapeutic goals of remission induction were defined as fulfilling each of the following after the 6-month remission induction stage: (1) ≥ 50% decline in the IgG4-RD responder index (RI); (2) GC tapered to maintenance dose; and (3) no relapse during GC tapering. The patients not achieving the therapeutic goals were considered to have failed of remission induction.ResultsThere were 26 patients in our cohort who failed of remission induction, including 16 (20.8%) on GC monotherapy, and 10 (7.2%) on combination therapy comprising GC and IM. The lacrimal gland and lung were most common sites of remission induction failure. Among the patients who relapsed during remission induction stage, 52.9% had secondary relapse during follow-up. Eosinophilia, higher baseline RI, more than five organs involved and dacryoadenitis were risk factors for remission induction failure with GC monotherapy, and the incidence of remission induction failure was 71.4% in the patients with more than three risk factors. After 6-month treatment, the patients who failed of remission induction had significantly higher erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and IgG4.ConclusionIn our cohort, 20.8% of patients failed of remission induction with GC monotherapy, while 7.2% of patients failed of remission induction with combination therapy comprising GC and IM.Electronic supplementary materialThe online version of this article (10.1186/s13075-018-1567-2) contains supplementary material, which is available to authorized users.

Efficacy and safety of iguratimod plus corticosteroid as bridge therapy in treating mild IgG4‐related diseases: A prospective clinical trial

“…In addition, there are some studies published in Chinese which showed iguratimod treatment was effective for primary Sjögren's syndrome and patient's serum Ig was decreased as well. As we know, one of the most prominent characteristics of IgG4‐RD is activation of B cells and plasmablast cells, which excrete large amount of serum IgG and IgG4 . Therefore, we think that iguratimod treatment may be effective in the treatment of IgG4‐RD.…”

“…Immunoglobulin G4‐related disease (IgG4‐RD) is a newly defined chronic fibro‐inflammatory disorder characterized by elevated serum IgG4 levels, tumefactive lesions with a dense lymphoplasmacytic infiltration rich in IgG4 positive plasma cells and storiform fibrosis of related organs . Glucocorticoids are the first‐line agents for the treatment of IgG4‐RD; however, in order to maintain long‐term disease stability and avoid disease relapse, usually glucocorticoids maintenance therapy should last for a long period, which may induce various glucocorticoid‐associated adverse reactions. For some IgG4‐RD patients with mild symptoms, such as swelling of the lacrimal glands, submandibular glands, parotid glands and nasal sinus, without internal organ damage, long‐term glucocorticoids therapy for mild symptoms may have a low benefit/risk ratio.…”

Identifying clinical subgroups in IgG4-related disease patients using cluster analysis and IgG4-RD composite score

“…The initial 6-month period was defined as the remission induction stage, and the therapeutic goals of this stage were defined as fulfilling each of the following: (1) ≥ 50% decline in the IgG4-RD RI, (2) GC tapered to maintenance dose (prednisone ≤ 10 mg/day), and (3) no relapse during GC tapering (within 6 months) [16].…”

“…An increasing number of studies on the outcomes and prediction of disease relapse in IgG4-RD showed that high baseline serum IgG4, IgE, and eosinophilia could predict IgG4-RD relapse independently [15]; eosinophilia, higher baseline RI, having five or more organs involved, and dacryoadenitis were risk factors for remission induction failure with GC monotherapy [16]; and hypocomplementemia was more frequent in IgG4related kidney disease (IgG4-RKD), which may participate in the disease development [17,18]. However, the disease subclass, outcomes, and predictors of disease relapse in IgG4-RD still require further exploration.…”

Acute unilateral periorbital edema

“…Recurrence rates for corticosteroid monotherapy range from 20.8% to 43%. Factors considered to be less favorable in terms of prognosis include high initial serum IgG4 levels and insufficient treatment duration [14,15]. Alternative treatment options include steroid-sparing immunosuppressants such as methotrexate, azathioprine or mycophenolate mofetil.…”