J Rheum Dis.  2013 Dec;20(6):364-368. 10.4078/jrd.2013.20.6.364.

A Case Report of Renal Involvement in Adult-Onset Still's Disease over Age 70

• Yoon GS
• Park W
• Baek JH
• Han JY
• Jung KH
• Lim MJ
• Joo K
• Kwon SR

• KMID: 2222995
• DOI: http://doi.org/10.4078/jrd.2013.20.6.364

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by spiking fever, arthralgia, salmon pink rash, neutrophilic leukocytosis, and multi-organ involvement. Although renal involvement may appear 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 39.0degrees 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.

Keyword

Adult-onset Still's disease; Renal involvement; Proteinuria

MeSH Terms

Adult
Arthralgia
Autoantibodies
Biopsy
Communicable Diseases
Diagnosis
Exanthema
Female
Fever
Fever of Unknown Origin
Glomerulonephritis
Glucocorticoids
Humans
Leukocytosis
Lymph Nodes
Lymphatic Diseases
Methotrexate
Neutrophils
Proteinuria
Rheumatic Diseases
Salmon
Still's Disease, Adult-Onset*
Autoantibodies
Glucocorticoids
Methotrexate

Figure

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  Figure 1. The chest CT shows paratracheal, paraaortic and subaortic lymph node enlargement.

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  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).

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  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.

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  Figure 4. Characteristics of the WBC, AST, ALT, ESR, CRP, LDH.

Reference

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