Journal List > J Rheum Dis > v.20(6) > 1064079

Yoon, Park, Baek, Han, Jung, Lim, Joo, and Kwon: A Case Report of Renal Involvement in Adult-Onset Still's Disease over Age 70

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by spiking fever, arthral-gia, salmon pink rash, neutrophilic leukocytosis, and mul-ti-organ involvement. Although renal involvement may ap-pear in some cases of adult Still's disease, onset over 70 years of age with renal involvement has not been described. We report a 73-years-old woman whose illness manifested with fever of unknown origin, massive proteinuria, and multiple lymph nodes enlargement. With proteinuria of 2,650 mg/day, a renal biopsy was performed, and histopathological evaluation yielded the diagnosis of chronic glomerulonephritis (CGN). After excluding infectious disease, malignancy, and other rheumatic disease, AOSD was diagnosed with symptoms including fever over 39.0 ° C for more than a week, leukocytosis, generalized lymphadenopathy, and negative autoantibodies. Proteinuria and fever were improved markedly by high dose glucocorticoids and methotrexate therapy.

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Figure 1.
The chest CT shows paratracheal, paraaortic and subaortic lymph node enlargement.
jrd-20-364f1.tif
Figure 2.
(A) The histologic section of the kidney shows enlarged glomerulus with mild mesangial proliferation and hyaline arteriolosclerosis (PAS, ×200). (B) The tubules are focally atrophic and interstitium shows focal fibrosis (masson trichrome, ×200).
jrd-20-364f2.tif
Figure 3.
Characteristics of the ferritin and proteinuria under therapy with prednisolone and methotrexate. The horizontal axis: date. The vertical axis: right, ferritin (ng/mL): left, proteinuria (mg/day). Reference ranges: ferritin, 13∼150 ng/mL; proteinuria, 21∼120 mg/day. MTX, methotrexate.
jrd-20-364f3.tif
Figure 4.
Characteristics of the WBC, AST, ALT, ESR, CRP, LDH.
jrd-20-364f4.tif
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