SAPHO Syndrome Diagnosis and Treatment: Report of Five Cases and Review of the Literature

Abstract

Background:The term “SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis) syndrome” includes a variety of musculoskeletal disorders associated with skin conditions; Osteitis is the most prominent skeletal lesion, whereas palmoplantar pustulosis and acne are the main skin lesions. Diagnosing SAPHO syndrome is difficult, because this syndrome is often confused with suppurative osteomyelitis, which has similar clinical and pathologic findings. SAPHO diagnosis is even more difficult when atypical sites are involved and there are no skin lesions.Patients and Methods:This case series presents five patients (3 women, 2 men), ages 27 to 44 years, who came to the Orthopaedic Department outpatient clinic for evaluation of pain in the humerus, clavicle, sacroiliac joints, and/or distal radius, and were diagnosed with SAPHO syndrome. Clinical and radiologic findings, treatment and outcome data, with up to 4 years of follow-up are presented. An extensive discussion of the clinical presentation, published literature, treatment options and outcome of SAPHO syndrome is also included.Results:Once the diagnosis of SAPHO syndrome was established, treatment with antibiotics (clindamycin) and non steroid anti-inflammatory drugs (lornoxicam) was remarkably effective. All patients did well and remained symptom free for up to four years, after a 3-8 month course of treatment.Interpretation:SAPHO syndrome should be included in the differential diagnosis when evaluating patients with lytic, sclerotic, or hyperostotic bone lesions and pain. Prompt SAPHO syndrome recognition, followed by appropriate therapy with antibiotics and NSAIDs can produce rapid symptom resolution, while avoiding unnecessary procedures and longterm antibiotic therapy.

Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report

“…Bacterial cultures are usually negative 2 but both Staphylococcus aureus and Propionibacterium acnes (P. acnes) have been isolated 11 in some cases, but the correlation between the pathogenesis and these agents are still controversial. Bone biopsy is often used in an attempt to establish a diagnosis, especially in cases with extraaxial bone lesions 12 . Bone histopathology is variable, with early lesions characterized by the presence of polymorphonuclear infiltrate.…”

“…Bone histopathology is variable, with early lesions characterized by the presence of polymorphonuclear infiltrate. Laboratory findings are usually of little value, and include mild, non-specific elevation of various inflammatory indices 12 . We evaluated our patient by MRI, and bone scintigraphy, but we needed a bone biopsy to rule out malignancy.…”

Intracranial manifestations in SAPHO syndrome: the first case report in literature

“…The role of infection in the etiology of SAPHO is debatable. While most reports in literature showed negative bacterial culture [7,8], Edlund et al [3] isolated P acne in six out of 11 patients with palmoplantar pustulosis. Treatment with antibiotics has been ineVective in most of the reports [1,9].…”

Cefcapene Pivoxil Hydrochloride Is a Potentially New Treatment for Palmoplantar Pustulosis with Pustulotic Arthro-Osteitis

“…SAPHO syndrome has been successfully treated with antibiotics, including azithromycin, doxycycline, minocycline, clindamycin and sulfamethoxazole-trimethoprim (table 1) [4,5,6,7,8,9,10,11,12]. The application of antibiotic therapy appeared to control the disease; however, disease relapse was observed after antibiotic discontinuation [10].…”

“…Similar to rheumatoid arthritis treatment, methotrexate and cyclosporine can be selected as further therapeutics for SAPHO joint pain. Antibiotics such as macrolides and tetracyclines have been used for the treatment of SAPHO syndrome in several reports [4,5,6,7,8,9,10,11,12]. There are a few options for the treatment of PPP or PPP with PAO to date, although antibiotics have been administered to several patients with PPP and PAO, referencing SAPHO treatment.…”