Long-term Clinical Outcomes in Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome.

OBJECTIVE: To assess the outcome of empirical therapeutic interventions for synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. METHODS: The clinical features and treatment outcomes of a cohort of 21 patients diagnosed with SAPHO in Western Australia were reviewed retrospectively. RESULTS: All 21 patients met published diagnostic criteria; 20 (95%) were Caucasian, and the median age was 47 years. The median follow-up was 6 years (range, 2 to 32 years). Three patients (14%) received no treatment; 18 (86%) required conventional synthetic disease-modifying antirheumatic drug (DMARDs). Thirteen (62%) had an initial good response to methotrexate; 8 relapsed and progressed to biologic DMARDs (bDMARDs) during a period of 14 years. Of the 13 recipients on a tumor necrosis factor inhibitor, 11 (85%) continued treatment for a median of 4 years (range, 1 to 14 years), whereas none of 3 recipients of interleukin 17/23 continued treatment (median, 4 months). Higher Physician Global Assessment scores (better outcomes) were observed in bDMARD recipients (mean, 7.06±2.24 [SD]) compared with non-bDMARD recipients (mean, 5.63±2.50; P=.1672) after a median of 3 years of therapy. CONCLUSION: This study describes the broad range of clinical manifestations in SAPHO, variable courses over time, and inconsistent outcomes with diverse empirical therapies. Moderately good long-term treatment outcomes were observed in most recipients of tumor necrosis factor inhibitor. Poorer outcomes were observed with bisphosphonates and interleukin 17/23 axis inhibitors; however, low numbers preclude robust comparison. Suboptimal treatment may be associated with poorer clinical outcomes and greater skeletal damage. TRIAL REGISTRATION: Australian and New Zealand Clinical Trials Registry: ACTRN12619000445178.

Perianal pseudoverrucous papules and nodules in Hirschsprung's disease: Rapid resolution with oral loperamide.

We report the case of a 2-year-old boy with Hirschsprung's disease who developed perianal pseudoverrucous papules and nodules subsequent to persistent diarrhea and stool leakage after Giardia infection. Bleeding from the papules resulted in iron deficiency anaemia requiring blood transfusion and iron infusion. Topical therapies used over 6 months were of limited benefit and colostomy was considered, but the condition completely resolved after commencement of oral loperamide. This demonstrates that perianal pseudoverrucous papules and nodules can be severe but are entirely reversible upon removal of the source of skin irritation.

Does the ingrowth surface make a difference? A retrieval study of 423 cementless acetabular components.

The effect of factors such as design, alloy and coating type on bony or fibrous tissue ingrowth was evaluated in a study of 423 retrieved cementless acetabular shells representing 16 shell designs. Small-beaded (250µm) porous coatings, either with or without hydroxyapatite (HA) coatings, proved to be the superior porous surface for bone ingrowth. Small-beaded shells that were Duofix coated had predominantly fibrous tissue ingrowth. In addition to bead size, alloy type and surface type have significant effect on bone ingrowth. In contrast, there is no significant association between bone ingrowth and time in situ, with most bone ingrowth occurring early. Although roughened, press-fit shells have acceptable clinical and Registry data, they showed some of the lowest ingrowth/ongrowth scores of all the shells tested.