BIOMAT Database Logo BIOMAT Database Logo

Tubulointerstitial nephritis with IgA kappa-positive plasma cells in a patient with primary Sjögren's syndrome and monoclonal gammopathy. 2023

Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Department of Nephrology, Japanese Red Cross Society Kyoto Daiichi Hospital, 15-749 Honmachi Higashiyamaku Kyoto-Shi, Kyoto, 606-0981, Japan. sono0823@koto.kpu-m.ac.jp.

This is a case report of a 69-year-old Japanese man who has been undergoing treatment for primary Sjögren's syndrome (pSS) since he was 62 years. A renal biopsy, which revealed diffuse and severe mononuclear cell infiltration in the tubulointerstitium, was performed because of progressive renal dysfunction. Immunostaining demonstrated most of the infiltrating cells to be IgA, kappa, CD38, and CD138 positive. Immunofixation blood test revealed IgA kappa-type M protein; however, bone marrow abnormalities or lymph node enlargements on examination or imaging, respectively, were not observed. Tubulointerstitial nephritis caused by monotypic plasmacytic infiltration in pSS, accompanied with a monoclonal gammopathy of undetermined significance (MGUS), was diagnosed. A treatment of prednisolone 40 mg/day was initiated, promptly improving the patient's serum creatinine levels from 3.0 to 1.5 mg/dl. The infiltrating cells in pSS-associated tubulointerstitial nephritis are generally polytypic plasmacytes and lymphocytes, but in the present case, monotypic plasmacytes were predominant. This case is remarkable and rare and can be considered a complication of pSS or MGUS. Since it may become a new disease entity, it is important to accumulate similar cases.

UI MeSH Term Description Entries
D007070 Immunoglobulin A Represents 15-20% of the human serum immunoglobulins, mostly as the 4-chain polymer in humans or dimer in other mammals. Secretory IgA (IMMUNOGLOBULIN A, SECRETORY) is the main immunoglobulin in secretions. IgA,IgA Antibody,IgA1,IgA2,Antibody, IgA
D008297 Male Males
D008998 Monoclonal Gammopathy of Undetermined Significance Conditions characterized by the presence of M protein (Monoclonal protein) in serum or urine without clinical manifestations of plasma cell dyscrasia. Benign Monoclonal Gammopathies,Monoclonal Gammapathy of Undetermined Significance,Monoclonal Gammopathies, Benign,Monoclonal Gammapathies, Benign,Benign Monoclonal Gammapathies,Benign Monoclonal Gammapathy,Benign Monoclonal Gammopathy,Monoclonal Gammapathy, Benign,Monoclonal Gammopathy, Benign
D009395 Nephritis, Interstitial Inflammation of the interstitial tissue of the kidney. This term is generally used for primary inflammation of KIDNEY TUBULES and/or surrounding interstitium. For primary inflammation of glomerular interstitium, see GLOMERULONEPHRITIS. Infiltration of the inflammatory cells into the interstitial compartment results in EDEMA, increased spaces between the tubules, and tubular renal dysfunction. Interstitial Nephritis,Nephritis, Tubulointerstitial,Tubulointerstitial Nephritis,Interstitial Nephritides,Nephritides, Interstitial,Nephritides, Tubulointerstitial,Tubulointerstitial Nephritides
D010265 Paraproteinemias A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. These cells frequently secrete a structurally homogeneous immunoglobulin (M-component) and/or an abnormal immunoglobulin. Gammapathy, Monoclonal,Gammopathy, Monoclonal,Monoclonal Gammopathies,Paraimmunoglobulinemia,Paraimmunoglobulinemias,Paraproteinemia,Plasma Cell Dyscrasias,Monoclonal Gammapathies,Monoclonal Gammopathy,Cell Dyscrasia, Plasma,Dyscrasia, Plasma Cell,Monoclonal Gammapathy,Plasma Cell Dyscrasia
D010950 Plasma Cells Specialized forms of antibody-producing B-LYMPHOCYTES. They synthesize and secrete immunoglobulin. They are found only in lymphoid organs and at sites of immune responses and normally do not circulate in the blood or lymph. (Rosen et al., Dictionary of Immunology, 1989, p169 & Abbas et al., Cellular and Molecular Immunology, 2d ed, p20) Plasmacytes,Cell, Plasma,Cells, Plasma,Plasma Cell,Plasmacyte
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D012859 Sjogren's Syndrome Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis. Sicca Syndrome,Sjogren Syndrome,Sjogrens Syndrome,Syndrome, Sicca,Syndrome, Sjogren's

Related Publications

Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Tubulointerstitial nephritis with monotypic lympho-plasmacytic infiltrates in a patient with primary Sjögren's syndrome accompanied by IgA-type monoclonal gammopathy.
December 2019, BMC nephrology,
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Interstitial nephritis with infiltration of IgG-kappa positive plasma cells in a patient with Sjögren's syndrome.
January 2004, Rheumatology (Oxford, England),
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
[An autopsied case of Sjögren's syndrome associated with IgA (kappa) monoclonal gammopathy and hypopituitarism].
April 1985, Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine,
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Tubulointerstitial nephritis with IgM-positive plasmacytoid large lymphocyte infiltration in a patient with primary biliary cirrhosis and Sjögren's syndrome.
July 2010, Clinical nephrology,
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Tubulointerstitial Nephritis with IgM-Positive Plasma Cells.
December 2017, Journal of the American Society of Nephrology : JASN,
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Overestimation of hemoglobin in a patient with an IgA-kappa monoclonal gammopathy.
April 2000, Archives of pathology & laboratory medicine,
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Marked hypocomplementemia and tubulointerstitial nephritis in a male patient with Sjögren's syndrome.
January 2004, Modern rheumatology,
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Tubulointerstitial Nephritis Cases With IgM-Positive Plasma Cells.
September 2020, Kidney international reports,
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
Monoclonal gammopathy and Sjögren's syndrome.
November 1980, Hospital practice,
Kazuhiro Sonomura, and Yuuki Oobayashi, and Misa Iimori, and Hiroko Yamauchi, and Mayuka Nakayama
[A case of Sjögren's syndrome associated with monoclonal IgA gammopathy and subcorneal pustular dermatosis].
April 1986, Ryumachi. [Rheumatism],
Copied contents to your clipboard!