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IgG4-related kidney disease--an update.

Kawano M, Saeki T - Curr. Opin. Nephrol. Hypertens. (2015)

Bottom Line: Clinically, decreased renal function, or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography or diffuse thickening of the renal pelvic wall, are typical presenting features.TIN with characteristic imaging findings is a typical manifestation of IgG4-RKD in the interstitium, while MGN is a representative manifestation of the glomerular lesions.Although IgG4 is a central feature of IgG4-RD, the recent discovery of IgG4-negative IgG4-RD raises questions about the causative role of the IgG4 molecule in this context.

View Article: PubMed Central - PubMed

Affiliation: aDivision of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan bDepartment of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan.

ABSTRACT

Purpose of review: IgG4-related disease (IgG4-RD) is a recently recognized systemic inflammatory disorder that can affect most organs/tissues such as sarcoidosis. The kidney is a frequently affected organ with tubulointerstitial nephritis (TIN), the representative lesion of IgG4-RD. This review focuses on the latest knowledge of IgG4-related kidney disease (IgG4-RKD).

Recent findings: A wide range of renal manifestations of IgG4-RD, that is TIN, membranous glomerulonephritis (MGN) and other glomerular lesions, and pyelitis, are collectively referred to as IgG4-RKD. Clinically, decreased renal function, or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography or diffuse thickening of the renal pelvic wall, are typical presenting features. Although a rapid response to corticosteroid therapy is a very important feature of IgG4-TIN, in cases in which renal function is moderately to severely decreased before therapy, only partial recovery of renal function is obtained.

Summary: TIN with characteristic imaging findings is a typical manifestation of IgG4-RKD in the interstitium, while MGN is a representative manifestation of the glomerular lesions. Although IgG4 is a central feature of IgG4-RD, the recent discovery of IgG4-negative IgG4-RD raises questions about the causative role of the IgG4 molecule in this context.

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Related in: MedlinePlus

A variety of patterns of multiple low-density lesions on contrast-enhanced computed tomography (CT). Upper: Contrast-enhanced CT scan shows bilateral diffuse patchy involvement. Middle: Contrast-enhanced CT scan shows multiple parenchymal low-density lesions including mass-like lesions protruding beyond the surface of the kidney (arrow). Lower: Contrast-enhanced CT scan shows a rim-like lesion of the kidney.
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Figure 1: A variety of patterns of multiple low-density lesions on contrast-enhanced computed tomography (CT). Upper: Contrast-enhanced CT scan shows bilateral diffuse patchy involvement. Middle: Contrast-enhanced CT scan shows multiple parenchymal low-density lesions including mass-like lesions protruding beyond the surface of the kidney (arrow). Lower: Contrast-enhanced CT scan shows a rim-like lesion of the kidney.

Mentions: A distinguishing feature of IgG4-TIN is its characteristic imaging findings frequently observed on computed tomography (CT) [18,22,23]. Contrast-enhanced CT is most useful in delineating the characteristics and distribution of the renal lesions. Multiple or solitary, round or wedge-shaped, parenchymal low-density lesions are common on CT [22,23]. Generally, solitary lesions are very rare, but if encountered, the suspicion of malignant tumour is high and often leads to nephrectomy [24]. In some cases, the lesions are well defined on contrast-enhanced CT, but can be ill defined in others, and in the latter, ‘diffuse patchy involvement’ is a more suitable description in extreme cases (Fig. 1). In addition, mass-like lesions protruding beyond the surface of the kidney, suggestive of tumour progression, may be detected in some cases (Fig. 1). Corresponding to the rim-like lesion of type 1 AIP, a rim-like lesion of the kidney is occasionally seen along a part of the renal capsule (Fig. 1) [22,23]. In addition to parenchymal lesions, renal pelvic lesions are sometimes encountered as a diffuse thickening of the renal pelvis wall with smooth intraluminal surface during systemic evaluation of IgG4-RD by CT [18,22,23].


IgG4-related kidney disease--an update.

Kawano M, Saeki T - Curr. Opin. Nephrol. Hypertens. (2015)

A variety of patterns of multiple low-density lesions on contrast-enhanced computed tomography (CT). Upper: Contrast-enhanced CT scan shows bilateral diffuse patchy involvement. Middle: Contrast-enhanced CT scan shows multiple parenchymal low-density lesions including mass-like lesions protruding beyond the surface of the kidney (arrow). Lower: Contrast-enhanced CT scan shows a rim-like lesion of the kidney.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4318645&req=5

Figure 1: A variety of patterns of multiple low-density lesions on contrast-enhanced computed tomography (CT). Upper: Contrast-enhanced CT scan shows bilateral diffuse patchy involvement. Middle: Contrast-enhanced CT scan shows multiple parenchymal low-density lesions including mass-like lesions protruding beyond the surface of the kidney (arrow). Lower: Contrast-enhanced CT scan shows a rim-like lesion of the kidney.
Mentions: A distinguishing feature of IgG4-TIN is its characteristic imaging findings frequently observed on computed tomography (CT) [18,22,23]. Contrast-enhanced CT is most useful in delineating the characteristics and distribution of the renal lesions. Multiple or solitary, round or wedge-shaped, parenchymal low-density lesions are common on CT [22,23]. Generally, solitary lesions are very rare, but if encountered, the suspicion of malignant tumour is high and often leads to nephrectomy [24]. In some cases, the lesions are well defined on contrast-enhanced CT, but can be ill defined in others, and in the latter, ‘diffuse patchy involvement’ is a more suitable description in extreme cases (Fig. 1). In addition, mass-like lesions protruding beyond the surface of the kidney, suggestive of tumour progression, may be detected in some cases (Fig. 1). Corresponding to the rim-like lesion of type 1 AIP, a rim-like lesion of the kidney is occasionally seen along a part of the renal capsule (Fig. 1) [22,23]. In addition to parenchymal lesions, renal pelvic lesions are sometimes encountered as a diffuse thickening of the renal pelvis wall with smooth intraluminal surface during systemic evaluation of IgG4-RD by CT [18,22,23].

Bottom Line: Clinically, decreased renal function, or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography or diffuse thickening of the renal pelvic wall, are typical presenting features.TIN with characteristic imaging findings is a typical manifestation of IgG4-RKD in the interstitium, while MGN is a representative manifestation of the glomerular lesions.Although IgG4 is a central feature of IgG4-RD, the recent discovery of IgG4-negative IgG4-RD raises questions about the causative role of the IgG4 molecule in this context.

View Article: PubMed Central - PubMed

Affiliation: aDivision of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan bDepartment of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan.

ABSTRACT

Purpose of review: IgG4-related disease (IgG4-RD) is a recently recognized systemic inflammatory disorder that can affect most organs/tissues such as sarcoidosis. The kidney is a frequently affected organ with tubulointerstitial nephritis (TIN), the representative lesion of IgG4-RD. This review focuses on the latest knowledge of IgG4-related kidney disease (IgG4-RKD).

Recent findings: A wide range of renal manifestations of IgG4-RD, that is TIN, membranous glomerulonephritis (MGN) and other glomerular lesions, and pyelitis, are collectively referred to as IgG4-RKD. Clinically, decreased renal function, or characteristic imaging findings such as multiple low-density lesions on contrast-enhanced computed tomography or diffuse thickening of the renal pelvic wall, are typical presenting features. Although a rapid response to corticosteroid therapy is a very important feature of IgG4-TIN, in cases in which renal function is moderately to severely decreased before therapy, only partial recovery of renal function is obtained.

Summary: TIN with characteristic imaging findings is a typical manifestation of IgG4-RKD in the interstitium, while MGN is a representative manifestation of the glomerular lesions. Although IgG4 is a central feature of IgG4-RD, the recent discovery of IgG4-negative IgG4-RD raises questions about the causative role of the IgG4 molecule in this context.

Show MeSH
Related in: MedlinePlus