<scp>IgG4</scp>‐related disease with cutaneous manifestations treated with rituximab: Case report and literature review

Jalilian, Chris; Prince, Miles; McCormack, Christopher; Lade, Stephen; Cheah, Chan Yoon

doi:10.1111/ajd.12100

Cited by 24 publications

(18 citation statements)

References 13 publications

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“…1b).IgG4-related disease is a newly described systemic, autoimmune, fibro-inflammatory condition that can present with cutaneous features, with recent reports demonstrating the effectiveness of multiple agents including oral steroids, mycophenolate mofetil, azathioprine, methotrexate, thalidomide and rituximab. 1,2,3,4,5 Our case complements that of Jalilian and colleagues and again demonstrates a late diagnosis after a previous parotidectomy, possibly because the condition was not well described when the patient initially presented.…”

supporting

confidence: 77%

Immunoglobulin Type Gamma 4‐Related Disease Presenting with Cutaneous Plaques

Bhabha

Turner

Bleasel³

et al. 2016

Aust J Dermatology

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We read with interest the recent article 'IgG4-related disease with cutaneous manifestations treated with rituximab: Case report and literature review 1 ' in Australian Journal of Dermatology and would like to report a case of extensive immunoglobulin type gamma 4 (IgG4)-related skin disease which responded extremely well to prednisolone and azathioprine.A 61-year-old woman presented at our clinic with a 3-year history of a large erythematous, nodular plaque on the left cheek ( Fig. 1a). Six months previously, similar lesions had developed on her forehead. Eight years prior, she had undergone a left parotidectomy and the histology revealed a non-specific lymphocytic infiltrate.A skin biopsy of the left cheek plaque demonstrated an inflammatory infiltrate of CD20-positive B-cells and CD4predominant T-cells, with a large number of IgG4 positive plasma cells. The parotid gland was re-examined, and histology demonstrated marked lymphoid hyperplasia with prominent germinal centres, polyclonal plasma cells and focal granulomas. Her serum IgG4 level was elevated at 2.24 g/L (0.03-2.01 g/L). A full body CT scan showed no systemic involvement. She was commenced on prednisolone and azathioprine with significant amelioration of the plaques (Fig. 1b).IgG4-related disease is a newly described systemic, autoimmune, fibro-inflammatory condition that can present with cutaneous features, with recent reports demonstrating the effectiveness of multiple agents including oral steroids, mycophenolate mofetil, azathioprine, methotrexate, thalidomide and rituximab. 1,2,3,4,5 Our case complements that of Jalilian and colleagues and again demonstrates a late diagnosis after a previous parotidectomy, possibly because the condition was not well described when the patient initially presented.

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supporting

confidence: 77%

Immunoglobulin Type Gamma 4‐Related Disease Presenting with Cutaneous Plaques

Bhabha

Turner

Bleasel³

et al. 2016

Aust J Dermatology

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“…Cutaneous involvement is rare but becomes increasingly appreciated, and Yamada and colleagues have reported that 6.3% of IgG4‐RD patients had cutaneous involvement . Skin lesions in IgG4‐RD typically present as erythematous papules and/or nodules that are commonly pruritic . It might be true that lung and/or skin can be the first target organs in patients with IgG4‐RD, but it is difficult, as well as challenging, to make the definitive diagnosis before the manifestations in other commonly‐affected organs such as the pancreas .…”

Section: Discussionmentioning

confidence: 99%

IgG4‐related disease manifesting the gastric wall thickening

Kawano

Ishii

Kimura

et al. 2015

Pathology International

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IgG4-related disease (IgG4-RD) is a recently designated disease entity and its full picture has not yet been elucidated. Here, we report an unusual case of a patient with gastric wall thickening secondary to IgG4-RD. A 68-year-old male visited our hospital with itchy skin lesions and an episode of organizing pneumonia. On the suspicion of malignancy-associated skin lesions, computed tomography (CT) was performed. The CT revealed prominent thickening of the gastric wall. Due to the possibility of malignancy, the patient underwent distal gastrectomy. Histopathological examination showed fibrosis of the submucosa and prominent thickening of the muscularis propria. Most of infiltrating cells were IgG4-positive plasma cells. Post-operative blood test revealed significantly high serum levels of total IgG and IgG4. Based on these histological features, the patient was given a definitive diagnosis of IgG4-RD. Further accumulation of cases like the present case that develop IgG4-RD with rare manifestations would lead to the elucidation of pathogenesis.

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“…11 Conventional steroid-sparing agents like mycophenolates, azathioprine, or methotrexate have all been used in treatment of IgG4-RD, but no case series or clinical trials supporting their efficacy and long-term outcomes exist. 12 Although limited studies available, radiotherapy has been suggested to be effective in steroid refractory IgG4-ROD. 13 Rituximab, a monoclonal antibody directed against the B lymphocytic-specific antigen CD20, might target the subset of plasma cells that can produce IgG4 antibodies in cases of IgG4-RD.…”

Section: Discussionmentioning

confidence: 99%

Infliximab for IgG4-Related Orbital Disease

Karim

Paridaens

Westenberg

et al. 2017

Ophthalmic Plastic &Amp; Reconstructive Surgery

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IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition with unclear pathophysiology. It may occur as a single organ disorder, but multiorgan presentation is common and can mimic several conditions. The preferred therapy consists of steroids, but definite maintenance strategy remains unclear. The authors describe a case of a 61-year-old woman, initially diagnosed with idiopathic orbital inflammation refractory to multiple immunosuppressive agents. The disease was complicated with epilepsy, vision loss, and trismus. Treatment with various immunosuppressive agents was unsuccessful. Eventually the patient was effectively treated with infliximab. This is the second case of IgG4-RD treated with a TNF-blocker documented in literature and the first description to demonstrate its superiority over steroid sparing agents. Although speculative, TNF-blockers might exert their effect in IgG4-RD by interfering with the possible overexpressed TNF alpha due to fibrosis in this disease. Treatment with infliximab appears a good alternative for refractory IgG4-RD. However, further studies are required to define the value of infliximab in IgG4-RD.

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IgG4‐related disease with cutaneous manifestations treated with rituximab: Case report and literature review

Cited by 24 publications

References 13 publications

Immunoglobulin Type Gamma 4‐Related Disease Presenting with Cutaneous Plaques

Immunoglobulin Type Gamma 4‐Related Disease Presenting with Cutaneous Plaques

IgG4‐related disease manifesting the gastric wall thickening

Infliximab for IgG4-Related Orbital Disease

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