Erdheim Chester disease: a rare cause of knee and leg pain

Sistermann, R.; Katthagen, B.-D.

doi:10.1007/pl00021229

Cited by 8 publications

(4 citation statements)

References 4 publications

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“…Treatment for ECD includes steroids, cytotoxic agents (3–11), and double autologous hematopoietic stem cell transplantation (12). The efficacy of these treatments is difficult to determine, because many of them have been used in few patients or in combination with other drugs and/or with a short followup period.…”

mentioning

confidence: 99%

Variability in the efficacy of interferon‐α in Erdheim‐Chester disease by patient and site of involvement: Results in eight patients

Haroche¹,

Amoura²,

Trad³

et al. 2006

Arthritis & Rheumatism

154

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Objective. Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown origin, characterized by infiltration of tissues by spumous histiocytes. ECD features heterogeneous systemic manifestations, and the general prognosis remains poor despite various treatment options.Methods. We treated 8 patients with multisystemic ECD with subcutaneous interferon-␣ (IFN␣) at a dosage of 3-9 ؋ 10 6 units 3 times weekly, for a median duration of 23 months (range 1-46 months).Results. Treatment was generally well tolerated, and side effects remained limited to fever following injections. Treatment was discontinued in 1 patient, because of severe depression. During treatment, some manifestations of ECD disappeared (i.e., xanthelasma, exophthalmos, papilledema, and intracranial hypertension). The efficacy of IFN␣ on cardiovascular ECD was variable, however. Treatment resulted in partial regression of "coated aorta" in some cases and clear failure in others; 2 patients died. The level of C-reactive protein diminished sharply in 5 patients.

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mentioning

confidence: 99%

Variability in the efficacy of interferon‐α in Erdheim‐Chester disease by patient and site of involvement: Results in eight patients

Haroche¹,

Amoura²,

Trad³

et al. 2006

Arthritis & Rheumatism

154

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“…In der Literatur finden sich bisher mehr als 50 Fallbeschreibungen mit histologischer Befundsicherung. Männer sind häufiger betroffen als Frauen (11). Der Altersgipfel liegt in den mittleren Lebensjahren (21-76 Jahre, Durchschnittsalter 54 Jahre) (7).…”

Section: Diskussionunclassified

“…Zentralnervöse Beteiligungen der ECD sind in ca. 1/3 aller Fälle beschrieben worden (7,9,11,15). Die klinische Manifestation ist mannigfaltig, da prinzipiell alle Areale des ZNS involviert sein können.…”

Section: Diskussionunclassified

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Erdheim-Chester-Krankheit

Koziolek

Kunze²,

Müller³

et al. 2005

Dtsch med Wochenschr

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The Erdheim-Chester syndrome is considered to belong to diseases with a proliferation of the monocytic-histiocytic and dendritic cellular system. In the presence of symmetric musculoskeletal symptoms associated with osteosclerotic and osteolytic lesions particularly occurring in the long bones of the lower extremities and concomitant with elevated serum markers of inflammation, the Erdheim-Chester disease should be taken into account. To date, no validated therapy exists.

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Lipidoses, Histiocytoses, and Hyperlipoproteinemias

Resnick¹,

Kransdorf²

2005

Bone and Joint Imaging

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Erdheim Chester disease: a rare cause of knee and leg pain

Cited by 8 publications

References 4 publications

Variability in the efficacy of interferon‐α in Erdheim‐Chester disease by patient and site of involvement: Results in eight patients

Variability in the efficacy of interferon‐α in Erdheim‐Chester disease by patient and site of involvement: Results in eight patients

Erdheim-Chester-Krankheit

Lipidoses, Histiocytoses, and Hyperlipoproteinemias

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