The case presented here has the unique feature of adrenal deficiency presenting alongside the SAPHO syndrome and is presented as the first case reported. [casesjournal.biomedcentral.com]

Presentation in the pediatric population is not uncommon. [radiopaedia.org]

Subligamentous thickening was present in 12 (67%). Paraspinal soft-tissue involvement was present in 6 (33%). [ajnr.org]

The diagnosis is made on clinical presentation, however the role of imaging studies is invaluable [4]. [termedia.pl]

The affected individuals usually present with pain, swelling, and limitations of movement in the affected joints. [ncbi.nlm.nih.gov]

• Pain

  Pain within the joint is a common cause of shoulder pain, ankle pain, and knee pain. Joint pain is also referred to as arthralgia. The sexually transmitted diseases (STDs) chlamydia and gonorrhea can lead to joint pain. [medicinenet.com]

  CASE PRESENTATION A 61 years old woman presented to our Pain Therapy and Palliative Care Unit with severe axial pain and gait impairment. [scitcentral.com]

  Although the skin lesions improved, after eight months, the back pain worsened. The patient was switched to adalimumab 40 mg every other week. He continues to require narcotic analgesics for pain relief. [the-rheumatologist.org]

  She complained of permanent lumbar spine pain increased by direct pressure on L1 and L5, with radiation of pain in the left buttock, and marked tenderness over the left sacroiliac joint. [jamanetwork.com]

• Skin Disease

  SAPHO syndrome is suspected when a patient presents with a pustular skin disease in association with rheumatic pain. [dermnetnz.org]

• Arthritis

  […] or acne-associated SpA, and hidradenitis suppurativa–associated arthritis. [rheumaknowledgy.com]

  The arthritis usually affects the lower limbs; it may be acute and polyarticular. [medical-dictionary.thefreedictionary.com]

  Pain is also a feature of joint inflammation (arthritis, such as rheumatoid arthritis and osteoarthritis) and infection, and extremely rarely it can be a cause of cancer of the joint. [medicinenet.com]

  1 Skin inflammation associated with arthritis, synovitis and enthesitis. Part 1: psoriatic arthritis, SAPHO syndrome, Still's disease, Behçet's disease. ( 30958944 ) 2019 2 Skin inflammation associated with arthritis, synovitis and enthesitis. [malacards.org]

  The most common site of the disease is the upper anterior chest wall, characterized by predominantly osteosclerotic lesions, hyperostosis, and arthritis of the adjacent joints. [pubs.rsna.org]

• Ankylosis

  […] sternoclavicular joint, osteitis of the medial end of the clavicle, first rib and sternum as well as hypertrophy of the costal cartilages osteitis, hyperostosis and hypertrophy of the medial ends of the clavicles, sternum and upper ribs and ultimately ankylosis [radiopaedia.org]

  Bony ankylosis is uncommon. Spondylodiscitis is the most common axial finding and is suggested by erosions of the vertebral plates with reactive vertebral sclerosis. Sacroiliitis is seen in as many as one-third of patients. [rheumaknowledgy.com]

  There have been multiple reports of mandible and temporomandibular joint involvement presenting with recurrent pain, swelling and limitation of movement at the jaw progressing to ankylosis [ 11, 12, 13 ]. [ncbi.nlm.nih.gov]

• Enthesopathy

  Also patients with SAPHO syndrome of the sternoclavicular joint often have painless enthesopathy as determined by ultrasound at sites remote from the lesions [2]. [enthesis.info]

  Part 1: psoriatic arthritis, SAPHO syndrome, Still's disease, Behçet's disease. ( 30615277 ) 2019 3 SAPHO syndrome with enthesopathy. ( 30617190 ) Su CF...Tsai CY 2019 4 Monoclonal Gammopathy of Undetermined Significance in a Patient With Synovitis, Acne [malacards.org]

  Enthesopathy can lead to ligament ossification, which can result in the development of bony bridging across joints. [ncbi.nlm.nih.gov]

  However, some authors maintain that the SAPHO syndrome can be regarded as a seronegative spondyloarthropathy due to the presence of high incidence of sacroiliitis, enthesopathy, axial involvement, inflammatory bowel disease and HLAB27 carriage and the [casesjournal.biomedcentral.com]

• Arthralgia

  A rare association of severe acne and arthralgia/arthritis, which is more common in men. Clinical findings The acne affects the face, upper back and trunk, and is accompanied by fever and weight loss. [medical-dictionary.thefreedictionary.com]

• Oligoarthritis

  Synovitis mostly manifests as nonerosive oligoarthritis of larger joints. Joint involvement can be primary arthritis or an extension of the osteitis adjacent to the articular structures. Arthritis has been reported in up to 92.5 % of SAPHO cases. [ncbi.nlm.nih.gov]

Laboratory workup included ESR, CRP and CBC and was normal. Femur X-Rays showed one osteosclerotic lesion with cortical thickening and periosteal new bone formation (Fig. 1 ). [ncbi.nlm.nih.gov]

Symptoms improved significantly within 3 months after treatment started. After continuing treatment for eight months, right sterno-clavicular joint swelling and tenderness had disappeared, and treatment stopped. [ncbi.nlm.nih.gov]

Treatment with oral and subsequently parenteral bisphosphonates and etanercept were all ineffective. [the-rheumatologist.org]

Treatment of SAPHO syndrome typically involves medications which reduce inflammation. [assignmentpoint.com]

Treatment of fibrous dysplasia of bone with intravenous pamidronate: long-term effectiveness and evaluation of predictors of response to treatment. Bone. 2004; 35 235-242 4 Kirchhoff T, Merkesdal S, Rosenthal H. et al. [thieme-connect.com]

The treatment with methotrexate (dose 25 mg/week) and non-steroidal anti-inflammatory drugs was effective. [termedia.pl]

Treatment and prognosis Tends to be benign although patients may have recurrent bouts of attacks. Pamidronate therapy has been successfully trialled for symptom relief (especially in the pediatric population) 6. [radiopaedia.org]

See Answer What is the outlook (prognosis) for patients with SAPHO syndrome? To some degree, the outlook for patients with SAPHO syndrome is not predictable but depends on response to medications. [medicinenet.com]

The generally good long term prognosis also suggests that innate immunity rather than autoimmunity is dominant in SAPHO [3] Diagnosis Painful swelling and tenderness of the breastbone and collar bone junction region is evident. [enthesis.info]

The treatment is difficult and often inadequate, despite good prognosis, involvement of multiple organ systems could complicate the disease course [ 4 ]. [casesjournal.biomedcentral.com]

The disease prognosis may be variable, presenting as a chronic stable or a relapsing-remitting course. In our case, each flare determined a progressive decline of quality of life with persistent pain and reduced mobility. [scitcentral.com]

Several bacteriological, immunological, and genetic mechanisms have been proposed as the etiology and thought to be involved in the pathogenesis of the SAPHO syndrome; however, to this day, the exact etiology remains unclear [ 4, 5 ]. [casesjournal.biomedcentral.com]

The etiology of this syndrome remains unclear but infectious, immunological and genetic factors are involved. The diagnostic features of SAPHO syndrome are clinical and radiological. [ncbi.nlm.nih.gov]

Etiology: The cause is unknown. SAPHO syndrome is often considered to be reactive owing to pustulosis, osteitis, and clinical similarities with the SpA. Pathology: Hyperostotic bony lesions, osteitis, and inflammatory synovitis are seen. [rheumaknowledgy.com]

Etiology and pathophysiology of the disease are not fully understood, but genetic, immunological and infectious factors are underlined [2, 3]. [termedia.pl]

However, as SAPHO syndrome etiology and pathophysiology are unknown, these treatment options are complex, uncomfortable, associated with multiple side effects [ 13, 15 ] and ineffective in curing the disease [ 16 - 18 ]. [ncbi.nlm.nih.gov]

Is it possible to prevent SAPHO syndrome? Because the tendency toward developing SAPHO syndrome is inherited, it is not possible to prevent the disease at this time. [medicinenet.com]

This regimen is a key factor to prevent the adverse effects of chronic NSAID therapy. [scitcentral.com]

In all but one patient pamidronate was effective in preventing recurrent bouts of pustulosis. Conclusion. [academic.oup.com]

Recognizing the disease in time will prevent osteoarticular progression. Otherwise, patients can suffer deformity, loss of function, and increasing pain, which might require wide resections. [ncbi.nlm.nih.gov]