SUN-326 CLINICAL MANIFESTATIONS AND LONG-TERM OUTCOMES OF IGG4-RELATED KIDNEY AND RETROPERITONEAL INVOLVEMENT IN A UNITED KINGDOM IGG4-RELATED DISEASE COHORT

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.

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Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

Scientific Reports ◽

10.1038/s41598-021-87718-6 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Jeong-Seon Lee ◽

Joong-Gon Kim ◽

Soyoung Lee

Keyword(s):

Polyarteritis Nodosa ◽

Clinical Manifestations ◽

Long Term Outcomes ◽

Childhood Onset ◽

Laboratory Findings ◽

Clinical Presentations ◽

Long Term Prognosis ◽

Digital Amputation

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

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Clinical Manifestations of Orbital Involvement in IgG4-Related Disease: A Retrospective Study of 573 Patients

10.21203/rs.3.rs-712045/v1 ◽

2021 ◽

Author(s):

Linyang Gan ◽

Xuan Luo ◽

Yunyun Fei ◽

Linyi Peng ◽

Jiaxin Zhou ◽

...

Keyword(s):

Lacrimal Gland ◽

Clinical Laboratory ◽

Clinical Manifestations ◽

Chinese Patients ◽

Laboratory Findings ◽

Male Predominance ◽

Orbital Involvement ◽

Related Disease ◽

Igg4 Related Disease ◽

Serum Igg4

Abstract Purpose: To investigate the clinical manifestations of orbital involvement in a large cohort of Chinese patients with IgG4-related disease (IgG4-RD). Methods: A total of 573 patients with IgG4-related disease were included. We described and compared the demographic, clinical, laboratory and histopathologic findings from 314 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 259 with extra-ophthalmic IgG4-RD.Results: Male predominance was found significant in extra-ophthalmic IgG4-RD only. Patients with IgG4-ROD showed younger age at diagnosis and longer duration from onset till diagnosis. In patients with extra-ophthalmic IgG4-RD, the most commonly involved extra-ophthalmic organ was pancreas; while in IgG4-ROD patients, salivary gland was most frequently affected. Multivariate analysis exhibited IgG4-ROD was associated with allergy history, higher serum IgG4/IgG ratio, multiple organs involvement and sialoadenitis. Orbital images were reviewed in 173 (55.1%) IgG4-ROD patients. Fifty-one (29.5%) patients had multiple lesions. Lacrimal gland involvement was detected in 151 (87.3%) patients, followed by extraocular muscles (40, 23.1%), other orbital soft tissue (40, 23.1%) and trigeminal nerve (8, 4.6%). Biopsy was performed from various organs in 390 cases. A dense lymphoplasmacytic infiltration and fibrosis were the main feature in orbital specimens. Storiform fibrosis and obliterative phlebitis were absent in lacrimal gland.Conclusions: Lacrimal gland involvement was the most common orbital manifestation of IgG4-ROD. Patients with IgG4-ROD showed different characteristic in demographic, clinical, laboratory findings compared to patients with extra-ophthalmic IgG4-RD. These features might indicate potential differences in the pathogenesis of these two subgroups of IgG4-RD.

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Clinical and immunological variants of ANCA-associated systemic vasculitis: a look at the phenomenon of IgG4 overproduction. Literature review and own observations

Rheumatology Science and Practice ◽

10.47360/1995-4484-2020-550-559 ◽

2020 ◽

Vol 58 (5) ◽

pp. 550-559

Author(s):

T. V. Beketova ◽

N. V. Kokosadze

Keyword(s):

Clinical Case ◽

Plasma Cells ◽

Systemic Vasculitis ◽

Clinical Manifestations ◽

Definite Diagnosis ◽

Related Disease ◽

Epitope Specificity ◽

Igg4 Related Disease ◽

Choice Of Treatment ◽

Combined Data

Systemic vasculitis (SV) associated with anti-neutrophilic cytoplasmic antibodies is a multifactorial process characterized by the variability of the epitope specificity of anti-neutrophilic cytoplasmic antibodies and the diversity of clinical phenotypes of the disease. In recent years, there has been an increasing interest in the combination of AAV and the phenomenon of IgG4 hyperproduction, which varies widely within AAV, from anti-neutrophilic cytoplasmic antibodies products of IgG4 subclass of undetermined significance, the presence of IgG4 positive plasma cells in the foci of immunoinflammatory lesions in patients with a definite diagnosis of AAV, to the typical clinical manifestations of an IgG4-related disease (IgG4-RD). We introduce own clinical case and analyze the combined data of the literature, which included 35 cases of a combination of AAV and IgG4-related pathology, indicating existing differences in the clinical manifestations of classical IgG4-RD and when combined with AAV. The currently accumulated data allows us to discuss the allocation of a specific clinical and immunological variant of AAV with IgG4 hyperproduction, characterized by a combination of clinical manifestations of AAV and clinical and / or histological signs of IgG4-related pathology. It is important to emphasize that the allocation of phenotypes of AAV in the future may be important for the personalized choice of treatment tactics for patients.

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Differences and similarities between IgG4-related disease with and without dacryoadenitis and sialoadenitis: clinical manifestations and treatment efficacy

Arthritis Research & Therapy ◽

10.1186/s13075-019-1828-8 ◽

2019 ◽

Vol 21 (1) ◽

Cited By ~ 5

Author(s):

Mu Wang ◽

Panpan Zhang ◽

Wei Lin ◽

Yunyun Fei ◽

Hua Chen ◽

...

Keyword(s):

Treatment Efficacy ◽

Clinical Manifestations ◽

Related Disease ◽

Igg4 Related Disease

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IgG4-related disease: long-term natural history and management of a relapsing multisystem disease entity

BMJ Case Reports ◽

10.1136/bcr-2017-219897 ◽

2017 ◽

pp. bcr-2017-219897 ◽

Cited By ~ 1

Author(s):

Sreelakshmi Kotha ◽

Giovanni Tritto ◽

Terry Wong ◽

Philip Berry

Keyword(s):

Natural History ◽

Disease Entity ◽

Multisystem Disease ◽

Related Disease ◽

Igg4 Related Disease

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Clinical outcomes and predictive relapse factors of IgG4‐related disease following treatment: a long‐term cohort study

Journal of Internal Medicine ◽

10.1111/joim.12942 ◽

2019 ◽

Vol 286 (5) ◽

pp. 542-552 ◽

Cited By ~ 6

Author(s):

Y. Peng ◽

J. Q. Li ◽

P. P. Zhang ◽

X. Zhang ◽

L. Y. Peng ◽

...

Keyword(s):

Cohort Study ◽

Clinical Outcomes ◽

Related Disease ◽

Igg4 Related Disease

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IgG4-Related Disease: A Reminder for Practicing Pathologists

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2017-0257-ra ◽

2017 ◽

Vol 141 (11) ◽

pp. 1476-1483 ◽

Cited By ~ 29

Author(s):

Steven C. Weindorf ◽

John Karl Frederiksen

Keyword(s):

Autoimmune Pancreatitis ◽

Plasma Cells ◽

Clinical Manifestations ◽

Organ System ◽

Related Disease ◽

Igg4 Related Disease ◽

Resection Specimen ◽

Histopathologic Features

IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic infiltrates rich in immunoglobulin (Ig) G4+ plasma cells. Initially characterized as a form of autoimmune pancreatitis, the distinctive histopathology of IgG4-RD has now been described in almost every organ system. However, because the clinical manifestations of IgG4-RD are diverse and nonspecific, the disease may go unsuspected until a biopsy or resection specimen is obtained to diagnose a presumed malignancy. Pathologists thus play a key role in the diagnosis of IgG4-RD, and familiarity with its histopathologic features is essential to preventing the irreversible comorbidities associated with this treatable disease. This brief review outlines the epidemiology, clinical manifestations, and histopathology of IgG4-RD, with the aim of furthering pathologists' awareness of and ability to diagnose this disorder.

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