zithromax and Acquired-Hyperostosis-Syndrome

In 1987, synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome was proposed as an umbrella term for a group of diseases with similar musculoskeletal manifestations, in particular hyperostosis of anterior chest wall, synovitis, and multifocal aseptic osteomyelitis, observed in association with dermatologic conditions such as palmoplantar pustulosis, severe acne, and hidradenitis suppurativa. Despite recent advances in our understanding of the epidemiologic, pathophysiologic, and immunogenetic mechanisms involved in SAPHO syndrome, etiopathogenesis remains poorly understood. Propionibacterium acnes, the microorganism associated with acne, has been recovered on bone biopsy in some patients, but the possible pathogenetic role of an infectious agent in a genetically predisposed individual, resulting in exaggerated inflammatory response as "reactive osteitis," is a largely unproven hypothesis. The newly available whole-body MRI will assist early diagnosis by detecting multifocal osteitis lesions, some of them asymptomatic, in axial (anterior chest wall, spine, and jaws) and nonaxial sites. Moreover, outcomes are vastly improved by treatment with bisphosphonates and tumor necrosis factor-alpha antagonists.

Topics: Acne Vulgaris; Acquired Hyperostosis Syndrome; Anti-Bacterial Agents; Azithromycin; Bone Density Conservation Agents; Diphosphonates; Humans; Hyperostosis; Magnetic Resonance Imaging; Osteitis

The acronym SAPHO was introduced in 1987 to unify the various descriptions of a seronegative arthritis associated with skin manifestations and to show synovitis, acne, pustulosis, hyperostosis, and osteitis with and without sterile multifocal osteomyelitis. The etiology of SAPHO syndrome is unknown, but an association with infection by semipathogenic bacteria like Propionibacterium acnes has been suggested. We conducted an interventional study of SAPHO patients receiving antibiotics.. Thirty-seven patients met the clinical criteria of SAPHO syndrome, 21 of them underwent a needle biopsy of the osteitis lesion, and 14 of them showed positive bacteriological cultures for P. acnes. Thirty patients (14 bacteriological positive and 16 without biopsy) were treated with antibiotics for 16 weeks. The activity of skin disease and osteitis were assessed by a physician using a scoring model (from 0 to 6). In addition, patients completed a Health Assessment Score (HAS, from 0 to 6). The erythrocyte sedimentation rate was determined and a MRI (of the osteitis lesion, radiologic activity score from 0 to 2) was performed in week 1 (W1), week 16 (W16), and week 28 (W28, 12 weeks after antibiotics).. Twenty-seven patients continued the medication (azithromycin, n = 25, 500 mg twice a week; clindamycin, n = 1, 300 mg daily; or doxycycline, n = 1, 100 mg daily) for 16 weeks. After W16 the scores for MRI (1.5 to 1.1, P = 0.01), skin activity (3.2 to 1.2, P = 0.01), osteitis activity (4.0 to 2.1, P = 0.02), and HAS (3.3 to 2.1, P = 0.01) decreased significantly. However, this was followed by increasing values for MRI scores (1.2 to 1.4, P = 0.08), skin activity (1.2 to 1.7, P = 0.11), osteitis activity (1.9 to 2.7, P = 0.01), and HAS (2.2 to 3.3, P = 0.02) from W16 to W28. The comparison of the scores in W1 and W28 in these 12 patients showed no significant differences.. For the period of application, the antibiotic therapy seems to have controlled the disease. After antibiotic discontinuation, however, disease relapse was observed. SAPHO syndrome thus groups with other chronic inflammatory arthropathies with a need for permanent therapy.

Topics: Acquired Hyperostosis Syndrome; Adult; Aged; Anti-Bacterial Agents; Azithromycin; Clindamycin; Doxycycline; Female; Gram-Positive Bacterial Infections; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Propionibacterium acnes; Treatment Outcome; Young Adult

A 64-year-old man complained of pain and a symmetric swelling of the clavicles. There were no fever and chills. 27 years earlier, a resection of the mandible was necessary, due to osteomyelitis. In the past, he complained of recurrent episodes of pain in the sternum and in the thoracic spine. Skin disorders were not reported.. The leucocyte count was within the normal range, however, levels of c-reactive protein and the erythrocyte sedimentation rate were increased. Autoantibodies and blood cultures were negative. On X-ray, osteosclerotic and cystic lesions in both clavicles were found, consistent with osteomyelitis. A bone scintigraphy revealed increased radionuclide activity in the clavicles and the sternum. A bone biopsy sample from the clavicle revealed signs of osteitis with fibrosis together with CD 68 und CD 138 positive cells. These findings indicated the diagnosis of a SAPHO syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) without skin disease.. NSAIDs, bisphosphonates and azithromycin were administered , thereby sufficient pain relief was rapidly achieved.. SAPHO syndrome is a rare disease complex including osteoarticular disorders which are frequently accompanied by skin disease. However, the diagnosis may be difficult due to a variable clinical manifestation and especially - like in our case - if skin disease is absent. Current treatment options are not evidence-based due to the rarity of the syndrome but include NSAIDs, bisphosphonates and glucocorticoids.

Topics: Acquired Hyperostosis Syndrome; Anti-Bacterial Agents; Anti-Inflammatory Agents, Non-Steroidal; Azithromycin; Clavicle; Diagnosis, Differential; Diphosphonates; Drug Therapy, Combination; Humans; Image Interpretation, Computer-Assisted; Imaging, Three-Dimensional; Male; Middle Aged; Osteomyelitis; Rare Diseases; Recurrence; Sternum; Thoracic Vertebrae; Tomography, X-Ray Computed

Topics: Acquired Hyperostosis Syndrome; Adolescent; Adult; Age Factors; Anti-Bacterial Agents; Azithromycin; Calcitonin; Child; Child, Preschool; Diagnosis, Differential; Diphosphonates; Female; Humans; Male; Middle Aged; Radionuclide Imaging; Time Factors

Topics: Acquired Hyperostosis Syndrome; Adult; Azithromycin; Calcitonin; Child; Chronic Disease; Diagnosis, Differential; Diphosphonates; Female; Humans; Osteomyelitis; Recurrence; Spinal Diseases

Topics: Acquired Hyperostosis Syndrome; Adult; Aged; Anti-Inflammatory Agents; Anti-Inflammatory Agents, Non-Steroidal; Azithromycin; Female; Glucocorticoids; Humans; Prednisone; Treatment Outcome