Showing papers by "Kazunori Yamada published in 2013"
TL;DR: IgG4-RD appears to have a distinctive clinicopathological presentation in the skin, differentiating it from other cutaneous disorders, and the occurrence of disease in other organs.
Abstract: IgG4-related skin disease is not widely recognized. This prompted us to investigate the clinical and pathological features of five patients we encountered with IgG4-related disease (IgG4-RD) affecting the skin. We investigated the clinical and pathological features of these five patients, including the distribution, onset, and morphology of eruptions, their pathological and immunohistochemical characteristics, and the occurrence of disease in other organs. The skin lesions were typically erythematous nodules and papules and brown papules like prurigo nodularis, which developed on the face or in the head and neck areas in four patients. Skin lesions were the first clinical manifestation in three patients. All five patients had sialadenitis and/or dacryoadenitis. The mean serum IgG4 concentration was 665.6 ± 410.0 mg/dl. Infiltrations of IgG4-positive plasma cells were observed in both the dermis and subcutaneous tissue. Germinal center formations were seen in three patients. Mild to moderate fibrosis was observed in all patients, and focal obliterative phlebitis in one. The average count of IgG4-positive cells was 67.3/high-power field (23.0–128.6). Wide variation in the numbers of infiltrating IgG4-positive cells was noted. IgG4-RD appears to have a distinctive clinicopathological presentation in the skin, differentiating it from other cutaneous disorders.
33 citations
TL;DR: A 75-year-old Japanese woman who had bilateral submandibular gland swelling, palpable purpura on the lower legs, and decreased renal function with hematoproteinuria and marked hypocomplementemia, but no skin lesion suggestive of systemic lupus erythematosus (SLE), and did not fulfill the classification criteria for SLE is reported.
Abstract: We report a case of Henoch- Schonlein purpura nephritis (HSPN) associated with tubulointerstitial nephritis (TIN) and chronic sclerosing sialoadenitis. The patient is a 75-year-old Japanese woman who had bilateral submandibular gland swelling, palpable purpura on the lower legs, and decreased renal function with hematoproteinuria and marked hypocomplementemia, but no skin lesion suggestive of systemic lupus erythematosus (SLE), and did not fulfill the classification criteria for SLE. Her serum IgG4 level was high and immunostaining of renal biopsies revealed marked infiltration by IgG4-positive plasma cells in the interstitium, confirming the diagnosis of IgG4- related disease. On the other hand, glomeruli showed endocapillary proliferative glomerulonephritis with mesangial IgA and C3 deposition demonstrated by immunofluorescence staining, which were typical glomerular lesions for HSPN. The glomerular and tubulointerstitial lesions responded to steroid therapy dramatically, and her renal function recovered to within the normal range within a month. This case suggests a possible new association between HSPN and IgG4-related disease.
30 citations
TL;DR: A patient with refractory cutaneous polyarteritis nodosa (CPAN) with hepatitis B virus (HBV) carrier status who was successfully treated with tumor necrosis factor alpha (TNF-α) blockade, using etanercept is described.
Abstract: We describe a patient with refractory cutaneous polyarteritis nodosa (CPAN) with hepatitis B virus (HBV) carrier status who was successfully treated with tumor necrosis factor alpha (TNF-α) blockade, using etanercept, and we review 5 similar cases. We administered etanercept because of the occurrence of repeated flares despite aggressive therapy. C-reactive protein normalization; prednisolone dose-sparing; and absence of any adverse events, including HBV reactivation with nucleotide analogue administration, or renal dysfunction, have been achieved for 8 months. TNF-α blockade should be considered for intractable CPAN.
22 citations
TL;DR: Analysis of regulatory role of FcγRIIB on the development of anemia using two additional IgG1 anti-RBC mAbs and different 34-3C IgG subclass-switch variants indicates that galactosylation, but not sialylated, of IgG 1 (but not other IgG subclasses) is critical for the interaction with F cγR, thereby determining the pathogenic potential of Igg1 autoantibodies.
20 citations
TL;DR: This case shows the possible involvement of Warthin's tumor with IgG4-RD, a 71-year-old man presenting with swollen right parotid and bilateral submandibular glands with history of autoimmune pancreatitis and an inflammatory abdominal aortic aneurysm.
14 citations
TL;DR: The renal lesions of the present two cases are quite different from each other, with one showing mildly atrophic kidneys with small numbers of cysts and the other an enlarged polycystic kidney disease, suggesting very heterogeneous entities.
Abstract: Background Situs inversus is a rare complication of cystic kidney diseases. Only three genes, INVS (NPHP2), NPHP3 and PKD2 have been proved to be responsible for some cases, while the responsible genes in many others are still unknown. Case reports Here we report two male patients with situs inversus combined with cystic kidney disease without any family history of polycystic kidney disease. Their renal function was normal in childhood but culminated in end stage renal disease in middle age. No pathogenic mutations were found in mutation analysis of INVS, IFT88, PKD2, UMOD or NPHP3 in them. Conclusions Past reported cases of situs inversus and cystic kidney diseases were divided into three groups, i.e., gestational lethal renal dysplasia group, infantile or juvenile nephronophthisis group and polycystic kidney disease group. The present patients are different from each of these groups. Moreover, the renal lesions of the present two cases are quite different from each other, with one showing mildly atrophic kidneys with small numbers of cysts and the other an enlarged polycystic kidney disease, suggesting very heterogeneous entities.
5 citations
TL;DR: A case of IgG4-related disease with Lacrimal and skin lesions, in which the skin lesions developed earlier than the lacrimal ones and resembled prurigo nodularis is reported.
Abstract: IgG4-related disease is a systemic inflammatory disease that affects multiple organs [1]. There have been only a limited number of reports describing skin lesions of IgG4-related disease, so the clinical and pathological characteristics of this condition remain unclear. Here, we report a case of IgG4-related disease with lacrimal and skin lesions, in which the skin lesions developed earlier than the lacrimal ones and resembled prurigo nodularis.A 47-year-old woman with a year-long history of a swollen [...]
5 citations
TL;DR: Clinical characteristics such as scanty subjective symptoms and much other organ involvement were identified in IgG4-related aortitis/periaortitis and periarteritis and the results suggest the necessity of systemic evaluation through radiographic examination not only at diagnosis but also during and after CS therapy in Igg4-RD.
Abstract: Background Recently, the new concept of IgG4-related disease (IgG4-RD) has been proposed [1], and it has been revealed that some chronic aortitis/periaortitis patients have an IgG4-related condition according to past studies examining surgically treated cases [2]. However, clinical characteristics and the course after corticosteroid (CS) therapy in patients with IgG4-related aortitis/periaortitis and periarteritis have not been well clarified. Objectives To clarify the clinical characteristics and the course after CS therapy in IgG4-related aortitis/periaortitis and periarteritis Methods We retrospectively evaluated clinical features including laboratory data, imaging findings and the clinical course after CS therapy in 20 patients diagnosed as having IgG4-related aortitis/periaortitis and periarteritis based on serum IgG4 levels, radiographic findings surrounding the artery, and extra-vascular IgG4-related organ involvement. Results Seventeen patients were men, and three were women (average age 66.3 years). Average follow-up period was 29.2 months (range 1-120 months). At diagnosis, their serum IgG4 concentration was 675 ± 541 mg/dL. Eighteen patients had extra-vascular organ involvement that fulfilled the diagnostic criteria of IgG4-RD [3]. Average number of organs involved was 3.7, with the most frequently affected organs being salivary glands (60%) and pancreas (40%). Concerning subjective signs/symptoms, only one patient showed low-grade fever, and no one complained of pain. The affected aorta/artery comprised 18 abdominal aortas, 4 thoracic aortas, 13 iliac arteries, one femoral artery, and two mesenteric arteries. Sixteen patients were treated with prednisolone (PSL) at an average initial dose of 29.3 mg/day (range 20-45 mg/day), and reduction in the size of the perivascular lesions was observed in 15 patients after therapy, although one patient showed relapse during PSL tapering at a dose of 7 mg/day. Five of 20 patients (25%) had aneurysm or slight lumen enlargement of the affected lesions at diagnosis. One of them was treated surgically. The other four patients were treated with PSL, in two (50%) of whom the aneurysm enlarged during the follow-up. New aneurysm formation was not observed in patients who had not had aneurysm of the affected lesions at diagnosis. Conclusions In the present study, clinical characteristics such as scanty subjective symptoms and much other organ involvement were identified in IgG4-related aortitis/periaortitis and periarteritis. Although the response to CS was good, some patients who had had aneurysm or slight lumen enlargement of the affected lesions at diagnosis showed aneurysm enlargement after CS therapy. These results suggest the necessity of systemic evaluation through radiographic examination not only at diagnosis but also during and after CS therapy in IgG4-RD. References Stone JH et al. IgG4-related disease.N Engl J Med. 2012;366(6):539-51. Kasashima S et al. IgG4-related inflammatory abdominal aortic aneurysm. Curr Opin Rheumatol. 2011;23(1):18-23. Umehara H et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21-30. Disclosure of Interest None Declared
2 citations
TL;DR: A relatively high percentage of IgG4-RD patients had persistent memory against tuberculosis, and Th2-mediated immune response to TB seems to be augmented in addition to Th1- mediated immune response in these patients.
Abstract: Background IgG4-related disease (IgG4-RD) is an inflammatory systemic disease, whose lachrymal and salivary gland involvement is known as Mikulicz’s disease and resembles that of primary Sjogren’s syndrome (pSS) 1) . In IgG4-RD, increased Th2 and regulatory immune reactions have been shown to be involved in the pathogenesis 2) . Although the etiology of IgG4-RD is still unknown, several reports indicate some link between IgG4-RD and latent tuberculosis (TB) infection 3,4) . Objectives To evaluate this possible link between IgG4-RD and TB. Methods 18 patients with IgG4-RD (Mikulicz’s disease 15, autoimmune pancreatitis 2, IgG4-related kidney disease 1) and 21 patients with pSS were included in this study. We compared the results of QuantiFERON TB-2G (QFT-2G) test, past history of TB disease, history of past contact with TB, serum immunoglobulin concentrations, and eosinophil count in IgG4-RD and pSS groups. Next, eosinophil count, serum IgG levels, serum IgG4 levels, serum IgE levels, allergic predisposition, and peripheral blood mononuclear cell (PBMC) cytokine mRNA production in response to stimulation with ESAT-6 and CFP-10 between QFT-2G (+) and QFT-2G (-) group with IgG4-RD were compared. Results 8 patients with IgG4-RD (44%) were QuantiFERON TB 2G-positive. In contrast, none of the pSS patients was QFT-2G positive, with this difference statistically significant (p=0.0007). Eosinophil count was significantly higher in IgG4-RD group (335/mm 3 ) than pSS group (75/mm 3 ). Between QFT-2G (+) and QFT-2G (-) group with IgG4-RD, serum IgE levels were significantly higher in QFT-2G (-) group than in QFT-2G (+) group (1465 U/ml versus 393 U/ml, p=0.027), and past history of TB was observed only in QFT-2G (+) group (63% versus 0%, p=0.016). PBMC IFN-γ and IL-4 mRNA production in response to stimulation with ESAT-6 and CFP-10 was significantly increased in QFT-2G (+) patients with IgG4-RD (n=8) than in QFT-2G (-) patients with IgG4-RD (n=4) (58.5 versus 5.4, p=0,027, 9.4 versus 0.24, p=0.014, respectively). In contrast, PBMC IL-6, IL-10, and TGF-β mRNA production in response to stimulation with TB related antigens were not different between the groups. Conclusions A relatively high percentage of IgG4-RD patients had persistent memory against tuberculosis, and Th2-mediated immune response to TB seems to be augmented in addition to Th1-mediated immune response in these patients. References Yamamoto M, Ohara M, Suzuki C, et al. Elevated IgG4 concentrations in serum of patients with Mikulicz’s disease. Scand J Rheumatol. 2004;33:432-3. Zen Y, Fujii T, Harada K, et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology. 2007; 45: 1538-46. Kawano M, Yamada K, Kakuchi Y, et al. A case of immunoglobulin G4-related chronic sclerosing sialadenitis and dacryoadenitis associated with tuberculosis. Mod Rheumatol 2009; 19, 87-90. Imai T, Yumura W, Takemoto F et al. A case of IgG4-related tubulointerstitial nephritis with left hydronephrosis after a remission of urinary tract tuberculosis. Rheumatol Int 2012[Epub ahead of print]. Disclosure of Interest None Declared
1 citations