Is osteonecrosis of the lunate bone an underestimated feature of systemic sclerosis? A case series of nine patients and review of literature.
Osteonecrosis of the lunate bone, also known as Kienböck's disease, is a very rare disease of unknown cause. Until today, only six cases of osteonecrosis of the lunate bone in patients with systemic sclerosis (SSc) have been reported in the literature. It is unknown whether these few cases reflect only a coincidence of two rare diseases or whether osteonecrosis of the lunate bone is a potential currently underestimated disease-associated feature of SSc. In this study, we report the clinical course of nine SSc patients with magnetic resonance imaging proven osteonecrosis of the lunate bone and discuss associated disease characteristics and potential underlying pathophysiological mechanisms. Overall, our observations suggest that osteonecrosis of the lunate bone is a frequent and so far under-recognized manifestation of SSc which might be linked to SSc-related vasculopathy. It is important to distinguish osteonecrosis of the lunate bone from wrist arthritis in SSc patients because the clinical treatment is different. In general, the clinical progression of osteonecrosis of the lunate bone seems to be slow in SSc patients. As most of the patients have only minor complaints, watchful waiting in combination with analgesic therapy seems to be a feasible treatment approach in most patients whether an operative intervention might be necessary in rapid progressive cases.
Department of Internal Medicine and Rheumatology, Justus-Liebig-University Giessen, Kerckhoff-Clinic Bad Nauheim, Benekestr. 2-8, Bad Nauheim 61231, Germany. Electronic address: m.frerix@kerckhoff-klinik.de.
Marc
M
Frerix
Department of Internal Medicine and Rheumatology, Justus-Liebig-University Giessen, Kerckhoff-Clinic Bad Nauheim, Benekestr. 2-8, Bad Nauheim 61231, Germany; Department of Radiotherapy, University Hospital Münster, Münster, Germany.
Kai
K
Kröger
Department of Trauma Surgery, University of Giessen and Marburg, Giessen, Germany.
Gabor
G
Szalay
Department of Internal Medicine and Rheumatology, Justus-Liebig-University Giessen, Kerckhoff-Clinic Bad Nauheim, Benekestr. 2-8, Bad Nauheim 61231, Germany.
Ulf
U
Müller-Ladner
Department of Internal Medicine and Rheumatology, Justus-Liebig-University Giessen, Kerckhoff-Clinic Bad Nauheim, Benekestr. 2-8, Bad Nauheim 61231, Germany.
Ingo Helmut
IH
Tarner
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D012667:Lunate Bone / Q000000981:diagnostic imaging*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010020:Osteonecrosis / Q000000981:diagnostic imaging / Q000209:etiology*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000000981:diagnostic imaging
Kienböck's disease; lunate bone; magnetic resonance imaging; osteonecrosis; scleroderma; systemic sclerosis