PubMed · 15406282
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P HEILMANN. 1950-01-25. [Bone struma].. https://pubmed.ncbi.nlm.nih.gov/15406282/
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CONTEXT: GH excess affects approximately 20% of the patients with McCune-Albright syndrome (MAS). MAS is caused by sporadic, postzygotic, activating mutations in the GNAS gene, which codes for the cAMP-regulating protein, G(s)alpha (gsp oncogene). These same mutations are found in approximately one third of the sporadic cases of acromegaly. OBJECTIVE: We examined efficacy of the GH receptor antagonist, pegvisomant, in controlling gsp oncogene-mediated GH excess and skeletal disease (fibrous dysplasia of bone) associated with MAS. SETTING AND PATIENTS: Five MAS patients with GH excess were treated with 20 mg/d sc injection of pegvisomant for 12 wk in a randomized, double-blind, placebo-controlled crossover study at the National Institutes of Health. MAIN OUTCOME MEASURES: The primary measure of efficacy was normalization of IGF-I. Secondary outcome measures were reduction in serum IGF binding protein-3 (IGFBP-3), improvement of fatigue and sweating, and reduction in markers of bone metabolism and bone pain. RESULTS: Combined mean changes in serum IGF-I at 6 and 12 wk were -236.4 ng/ml (53%, P < 0.005) and -329.8 ng/ml (62%, P < 0.001), respectively. IGFBP-3 decreased by 0.8 mg/liter (24%, P < 0.01) and 2.9 mg/liter (37%, P < 0.005), respectively. There were no significant changes in signs and symptoms of acromegaly or markers of bone metabolism and bone pain, nor was there a significant change in pituitary size. Retrospective comparison of the degree of control achieved with pegvisomant vs. other medications (long-acting octreotide +/- dopamine agonist) in the same group showed that the two regimens were similarly effective. CONCLUSIONS: Pegvisomant effectively reduced IGF-I and IGFBP-3 levels in gsp-mediated GH excess but had no effect on fibrous dysplasia.
Entheses are sites where tendons, ligaments, joint capsules, or fasciae attach to bone. Their function is to provide a mechanism for reducing stress at the bony interface by dissipating the biomechanical load acting on the bone. Enthesitis may occur in traumatic, endocrinologic, metabolic, degenerative, or inflammatory conditions and is a major symptom in patients with seronegative spondyloarthritis (SpA). The increasing interest in inflammation of the entheses associated with rheumatoid diseases has fundamentally changed our understanding of the clinical presentation, pathogenesis, and therapy of enthesitis. Conventional radiographs and computed tomography (CT) allow good evaluation of chronic changes of the entheses, such as soft-tissue calcification, erosions, and new bone formation. The method of first choice to evaluate acute enthesitis is magnetic resonance imaging (MRI), which depicts both soft-tissue changes and intraosseous abnormalities. The purpose of this overview is to discuss the MRI appearance of enthesitis in patients with SpA and to provide a morphologic survey of the predominantly affected entheses.