Mehul Jariwala

Bio: Mehul Jariwala is an academic researcher. The author has contributed to research in topics: IgG4-related disease & Anti-nuclear antibody. The author has an hindex of 3, co-authored 4 publications receiving 23 citations.

Orbital immunoglobulin-G4-related disease: case series and literature review.

Abstract: Immunoglobulin-G4-related disease (IgG4-RD) is a distinct group of diseases characterized by elevated serum IgG4 titres and infiltration of affected organs by IgG4-positive plasma cells. IgG4-RD can involve any ocular adnexal tissue. They have a distinct prognosis and pattern of tissue involvement and hence need to be differentiated from orbital lesions with similar clinicopathological features. We report three cases of IgG4-RD and review the literature extensively discussing various aspects of this novel entity.

IgG4-Related Orbital Inflammation Presenting as Unilateral Pseudotumor

Abstract: IgG4 related systemic disease (IgG4-RSD) has been recognised in the last few years. Orbital pseudotumor as a presentation of IgG4-RSD is one of the rare complaints encountered in pediatric population. It is an inflammatory condition of unknown etiology characterized by tumorous swelling of the organs, characteristic histopathologic changes and elevated IgG4: IgG plasma cells ratio. The disease is also characterized by involvement of varied organ systems. The authors describe a seven-year-old boy with orbital pseudotumor after two years of initial onset with waxing and waning course, steroid responsive lesion and biopsy suggestive of IgG4-RSD involving the extraocular soft tissue. Treatment with oral corticosteroids and Azathioprine produced a significant decline in the pseudotumor size. It is important for pediatricians to be aware of this condition as appropriate recognition and management is important to prevent long-term damage of the tissue involved. This is the first case of IgG4 related orbital pseudotumor reported from India.

A77: Clinical and Laboratory Features and Systemic Lupus Erythematous Disease Activity Index 2000 (SLEDAI 2K) at Onset and Follow Up in a Cohort of 109 Paediatric Lupus Patients From a Tertiary Level Centre in India

Abstract: Background/Purpose: Pediatric Rheumatology is a relatively new branch in India. To the best of our knowledge, this is the first paper that has studied SLEDAI 2K in 109 children with SLE at onset, 6 months and annually thereafter for a median follow up of 38 months. Methods: In our unit, disease activity of all children with lupus is captured on disease assessment proformas and completed every 3–6 months. This form captures demographics, clinical assessment, lab results and therapy. All paediatric lupus patients (diagnosed per the ACR criteria 1992) with disease onset <18 years seen at our unit from 2001 were included. The clinical and laboratory features at presentation and the SLEDAI 2K was captured at onset, 6 months and thereafter annually. We chose SLE 2K as an outcome measure as it captures new, recurrent and ongoing disease activity ([1]). The aims of the study were To detail the presentation of cSLE from India To study the SLEDAI 2K at onset and follow up To compare the data from India to that available from the West. available from the West. Results: Demographics: 109 patients identified, 90 were females. The median age at onset was 11.4 (3.4-18) years and median age at diagnosis 12.1 (3.5-19). The median duration of follow up was 38 months (3-152). Clinical presentation at onset: As in table 1. Nine children had MAS at onset. Table 1. Table 1. Feature % positive Constitutional fever 83 arthralgia 80 Fatigue 70 myalgia 55 weight loss 43 Lupus features malar rash 62 arthritis 60 alopecia 59 mucositis 56 photosensitivity 39 vasculitis 38 nephritis 35 lymphadenopathy 33 neurologic 17 effusion 14 Labs at onset: ANA positive in 97%, DsDNA in 80%, low complements seen in 84%. Anemia, leucopenia and thrombocytopenia present in 37, 31, and 28% respectively. APL antibodies positive in 28%. SLEDAI 2K at onset and follow up: Table 2 Table 2. SLEDAI 2K in a Cohort of cSLE Patients at Onset and 5 Year Follow Up disease duration months no of patients Median SLEDAI % pts with SLEDAI <4 SLEDAI Range 0 109 16 2 1 to 52 6 107 2 56 0 to 28 12 97 2 66 0 to 16 24 77 2 70 0 to 28 36 58 2 71 0 to 24 48 44 0 75 0 to 50 60 37 2 70 0 to 12 Median SLEDAI 2K 16 at onset and subsequently all median SLEDAIs were <4. Patients with inactive disease (SLEDAI 2K <4) ([1]) 70–75% after the 6 months. Nineteen patients had a new organ involvement 6 months after disease onset, eight nephritis. Follow up: 54 patients had no flare, 27–1 flare and 28 patients had 2–5 flares each. One patient died, no patient had renal failure/transplant. Seven patients had hypothyroidism and 1 had celiac disease. Comparison with Western data: Of 256 patients with cSLE described from Toronto, the mean SLEDAI was 13 at onset and 2.9 at one year, very similar to our study ([2]). The cohort from Israel studied 102 patients with a higher SLEDAI of 17 at onset, 7.6 at 5 years with 5 patients in renal failure ([3]). Conclusion: 80% of patients had fever and 33% had nephritis at onset. The disease burden at onset was high; median SLEDAI fell to <4 within 6 months and remained low through follow up. 17% had new organ involvement after 6 months. 50% had no disease flare. The disease burden is similar to that described in the West.

IgG4-related disease: a systematic review of this unrecognized disease in pediatrics.

Abstract: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with an unclear pathophysiological mechanism affecting different parts of the body. If untreated, the disease can lead to fibrosis and irreversible organ damage. IgG4-RD mostly has been described in adults, hence it is generally unknown among pediatricians. This systematic search of the literature provides an overview of all reports published on IgG4-RD in children in order to create awareness of IgG4-RD in pediatrics and to emphasize the broad clinical presentation of this disease. A systematic literature search of Embase, Medline, Web-of-Science, PubMed publisher, Cochrane and Google Scholar was performed for case reports on IgG4-RD in children. Of total 740 articles identified by the search, 22 case reports including 25 cases of IgG4-RD in children were found. The median age of the children was 13 years, of which 64 % were girls. IgG4-related orbital disease (44 %) and autoimmune pancreatitis type 1/IgG4-related pancreatitis (12 %) predominantly occurred. Less frequently, other manifestations as pulmonary manifestation, cholangitis and lymphadenopathy were also found. Almost all cases were histologically proven. Prednisone was the first choice of treatment leading to favorable clinical response in 83 % of the cases. Maintenance therapy with steroid sparing agents was required in 43 % of the cases needing therapy. Rituximab was successful in all 4 cases, whereas, the disease modifying rheumatic drugs (DMARDs) mycophenolate mofetil, azathioprine and methotrexate were effective in almost 50 % of the cases. IgG4-RD in children is a generally unknown disease among pediatricians, but several pediatric cases have been described. Prednisone is the first choice of treatment leading to disease remission in the majority of the cases. DMARDs and rituximab are alternative effective steroid sparing agents with more positive evidence for the latter.

IgG4-Related Ophthalmic Disease: Pooling of Published Cases and Literature Review

Abstract: In recent years, IgG4-related ophthalmic disease (IgG4-ROD) has emerged as a common cause of orbital inflammation, accounting for a substantial proportion of idiopathic orbital inflammation and lymphoid hyperplasia. The last pooled analysis of published cases was conducted in 2012, but a large number of new cases have been added to the literature since then. In this review, we present the demographic, clinical, histological, and treatment data for 172 published cases of biopsy-confirmed IgG4-ROD. Results are accompanied by a review of the relevant literature.

The treatment outcomes in IgG4‐related orbital disease: a systematic review of the literature

Abstract: IgG4-related disease (IgG4-RD) is an immune-mediated systemic fibro inflammatory disease. Treatment of IgG4-related orbital disease (IgG4-ROD) is often indicated to relieve the symptoms and to prevent complications. For IgG4-ROD, no international formal treatment guidelines are available and the optimal treatment strategy is uncertain. In this systematic review, we describe the efficacy of conventional and biologic disease-modifying antirheumatic drugs (DMARDs) in IgG4-ROD. A systematic search of Embase, Medline, Web-of-Science, PubMed publisher, Cochrane and Google Scholar was performed for treatment outcomes in IgG4-ROD. Relevant articles on treatment of IgG4-ROD were retrieved to last date of inclusion 3 January 2018. The following inclusion criteria were used: articles in English or English translation, studies evaluating the use of DMARDs (conventional and biologic) in the treatment of IgG4-ROD. Meta-analysis and review articles were excluded. A final selection after full-text evaluation was made by independent reviewers, based on treatment of IgG4-ROD with DMARDs and the availability of treatment outcomes. With this systematic review, we identified 35 studies and case reports/series on IgG4-ROD, describing 95 patients, treated with conventional and/or biologic DMARDs. The success of conventional DMARDs varies between 36% and 75% in patients with IgG4-ROD, while rituximab is successful in the majority (93%) of the patients. Based on this systematic review, rituximab is the most effective DMARD in IgG4-ROD, while the efficacy of conventional DMARDs is limited. We propose early initiation of rituximab in case of refractory and organ- or life-threatening disease.

IgG4-related inflammatory pseudotumor: A systematic review of histopathological features of reported cases.

Abstract: Objectives: There is marked inconsistency in reporting the key features of IgG4-related inflammatory pseudotumor (IPT) cases. We aimed to analyze the various aspects of IgG4-related IPTs and to test the performance of the consensus criteria for their diagnosis.Methods: PubMed database was searched for IgG4-related IPT cases. The data regarding patient demographics, clinical presentation, laboratory findings, histopathological features, and treatment response are extracted and are presented here in a descriptive manner.Results: The study included 40 papers describing the clinicopathological features of 83 IPTs in 80 patients. Seventeen cases were diagnosed on biopsies; while remaining were diagnosed on excision specimens. Among these, 50 cases were categorized as highly suggestive and 24 cases as probable for IgG4RD; while nine cases had insufficient histopathological evidence of IgG4RD. Two cases diagnosed on biopsies having insufficient evidence of IgG4RD showed partial or no response to steroids...

Fibrosis, gene expression and orbital inflammatory disease

Abstract: Background/aims To clarify the pathogenesis of fibrosis in inflammatory orbital diseases, we analysed the gene expression in orbital biopsies and compared our results with those reported for idiopathic pulmonary fibrosis. Methods We collected 140 biopsies from 138 patients (58 lacrimal glands; 82 orbital fat). Diagnoses included healthy controls (n=27), non-specific orbital inflammation (NSOI) (n=61), thyroid eye disease (TED) (n=29), sarcoidosis (n=14) and granulomatosis with polyangiitis (GPA) (n=7). Fibrosis was scored on a 0–3 scale by two experts, ophthalmic pathologists. Gene expression was quantified using Affymetrix U133 plus 2.0 microarray. Results Within orbital fat, fibrosis was greatest among subjects with GPA (2.75±0.46) and significantly increased in tissue from subjects with GPA, NSOI or sarcoidosis (p Conclusions A pathologist9s recognition of fibrosis in orbital tissue correlates well with increased expression of transcripts that are considered essential in fibrosis. Many transcripts implicated in orbital fibrosis have been previously implicated in pulmonary fibrosis. TED differs from other causes of orbital fat inflammation because fibrosis is not a major component. Marked fibrosis is less common in the lacrimal gland compared with orbital adipose tissue.