Acute Tubulointerstitial Nephritis in Rosai-Dorfman Disease Mimicking IgG4-related Disease

Membranous nephropathy (MN) with tubulointerstitial nephritis (TIN) is an established manifestation of immunoglobulin G4 (IgG4)-related disease (IgG4-RD). A pathological feature aiming to distinguish between primary and secondary MN is the presence or absence of glomerular staining for phospholipase A2 receptor (PLA2R), respectively. Isolated MN without TIN has been rarely reported in the context of IgG4-RD. This case report describes a patient with a history of MN successfully treated with steroids and cyclophosphamide, who, 3 years later, presented with unexplained exacerbation of diabetes mellitus due to IgG4-related autoimmune pancreatitis. Pancreatitis was treated, and diabetes improved after treatment with steroids. Based on the presence of isolated IgG4 glomerular capillary deposits along with negative staining for PLA2R and the metachronous appearance of autoimmune pancreatitis, MN was retrospectively classified as secondary to IgG4-RD. Isolated IgG4-positive/PLA2R-negative MN without TIN can be a prodrome of IgG4-RD, reminiscent of MN secondary to neoplasms.

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Rosai-Dorfman Disease of the Pancreas Shows Significant Histologic Overlap With IgG4-related Disease

The American Journal of Surgical Pathology ◽

10.1097/pas.0000000000001334 ◽

2019 ◽

Vol 43 (11) ◽

pp. 1536-1546 ◽

Cited By ~ 3

Author(s):

Jessica Tracht ◽

Michelle D. Reid ◽

Yue Xue ◽

Emilio Madrigal ◽

Juan M. Sarmiento ◽

...

Keyword(s):

Related Disease ◽

Igg4 Related Disease ◽

Rosai Dorfman Disease

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IgG4-Related Disease Manifesting as Hypocomplementemic Tubulointerstitial Nephritis: A Rare Case Report and Literature Review

Journal of Investigative Medicine High Impact Case Reports ◽

10.1177/2324709620952213 ◽

2020 ◽

Vol 8 ◽

pp. 232470962095221

Author(s):

Pradnya Brijmohan Bhattad ◽

David L. Joseph ◽

Eric Peterson

Keyword(s):

Kidney Disease ◽

Vision Loss ◽

Tubulointerstitial Nephritis ◽

Organ Damage ◽

Immunoglobulin G4 ◽

Related Disease ◽

Multiple Organs ◽

Igg4 Related Disease ◽

Rare Case Report ◽

Progression Of Kidney Disease

Immunoglobulin G4–related disease (IgG4-RD) is a chronic fibrosing inflammatory systemic disorder that has been recognized relatively recently in the medical literature. Little is known about the exact disease pathogenesis and epidemiology. IgG4-RD may be asymptomatic or may have minimal symptoms or involve multiple organs with overt symptoms. The different phenotypes of IgG4-RD can lead to delayed or incorrect diagnosis. We report the case of a 66-year-old male with coal worker’s pneumoconiosis who presented with progressive kidney disease and was diagnosed with tubulointerstitial nephritis due to IgG4-RD. The patient was noted to have progressive kidney disease, skin involvement, worsening interstitial lung disease, complete vision loss in the left eye, and retroperitoneal fibrosis. Serologic workup revealed elevated inflammatory markers, IgG4 and IgG1 levels, and hypocomplementemia. A tissue biopsy helped us establish a definitive diagnosis of IgG4-RD and initiate treatment with glucocorticoids to prevent further progression of kidney disease and other end-organ damage.

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Rosai-Dorfman disease: an overview

Journal of Clinical Pathology ◽

10.1136/jclinpath-2020-206733 ◽

2020 ◽

Vol 73 (11) ◽

pp. 697-705

Author(s):

Cassandra Bruce-Brand ◽

Johann W Schneider ◽

Pawel Schubert

Keyword(s):

Plasma Cells ◽

English Literature ◽

Significant Proportion ◽

Cervical Lymphadenopathy ◽

Molecular Profile ◽

Related Disease ◽

Igg4 Related Disease ◽

Rosai Dorfman Disease ◽

Neoplastic Process

ContextRosai-Dorfman disease is an uncommon histiocytic disorder most frequently presenting as bilateral cervical lymphadenopathy in children and young adults. Extranodal disease occurs in a significant proportion of patients. It has been recently classified as part of the ‘R group’ of histiocytoses by the Histiocyte Society in 2016. Cutaneous Rosai-Dorfman disease is regarded as a separate disease entity that falls into the ‘C group’ of histiocytoses according to this classification system. The pathogenesis was previously poorly understood; however, recent evidence demonstrating clonality in a subset of cases raises the possibility of a neoplastic process. A possible association with IgG4-related disease remains controversial.ObjectivesTo provide a comprehensive review of Rosai-Dorfman disease, including nodal, extranodal and cutaneous forms, with a particular emphasis on new insights into the possible clonal nature of the disease; to discuss the recently revised classification of the histiocytoses by the Histiocyte Society; and to summarise the findings from the literature regarding the controversial association with IgG4-related disease.Data sourcesThis review is based on published peer-reviewed English literature.ConclusionsClassic Rosai-Dorfman disease, which may be sporadic or familial, is considered a separate entity from cutaneous disease, which is reflected in the revised classification of histiocytoses. An increase in IgG4-positive plasma cells may be seen in Rosai-Dorfman disease. This finding in isolation is of limited significance and should be interpreted with caution. Studies investigating the molecular profile of the disease show that in at least a subset of cases the disease is a clonal process. The classification of Rosai-Dorfman disease is therefore likely to change as our understanding of the aetiopathogenesis evolves.

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