SAPHO syndrome

SAPHO Syndrome: A Rare Inflammatory Disorder

SAPHO syndrome, also known as Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis, is a rare inflammatory disorder that affects the bones, joints, and skin. The condition was first described in 1987 and is characterized by a combination of symptoms, including:

• Synovitis: Inflammation of the joints, leading to pain, stiffness, swelling, warmth, and redness.
• Acne: Pustular acne on the face, chest, or back.
• Pustulosis: Thick yellow blisters containing pus on the palms and soles.
• Hyperostosis: Increase in bone substance, leading to abnormal bone growth.
• Osteitis: Inflammation of the bones.

Key Features

• SAPHO syndrome is a rare disorder with a wide range of possible symptoms [2].
• The condition is more common in women than in men [3].
• Patients typically present with a combination of skin and joint symptoms, including acne, pustulosis, and synovitis.
• Osteitis and hyperostosis are also common features of the disorder.

References

[1] SAPHO syndrome involves any combination of: Synovitis (inflammation of the joints), Acne, Pustulosis (thick yellow blisters containing pus) often on the palms and soles, Hyperostosis (increase in bone substance) and Osteitis (inflammation of the bones). [Context 1]

[2] SAPHO syndrome is a rare disorder with a wide range of possible symptoms. Some common symptoms include blisters, acne, and inflammation of the joints and bones. [Context 2]

[3] SAPHO syndrome is more common in women than in men. [Context 3]

Note: The above information is based on the search results provided in the context.

SAPHO syndrome is a rare disorder that involves a combination of signs and symptoms affecting the joints, bones, and skin.

Common Signs and Symptoms:

• Synovitis (inflammation of the joints) [5]
• Acne (conglobata or fulminans) [3][6]
• Pustulosis (thick yellow blisters containing pus) often on the palms and soles [5]
• Hyperostosis (increase in bone substance) [4]
• Osteitis (inflammation of the bones) [4]

Other Potential Symptoms:

• Fatigue [12]
• Fever [12]
• Gastrointestinal symptoms such as abdominal pain or diarrhea [12]
• Eye inflammation (uveitis) [12]
• Oral ulcers [12]

Clinical Features:

• Morning stiffness is a common presenting sign [8]
• Soft tissue swelling, often with redness and heat, and limitation of motion at the affected skeletal site(s) are other presenting signs [8]
• Bony lesions manifest as severe, recurrent, debilitating pain [4][7]

Note that SAPHO syndrome can be challenging to diagnose due to its overlapping features with other inflammatory disorders. The diagnostic criteria for SAPHO syndrome were developed by Kahn et al. in 1994, but some authors suggest that it could be considered as a type of spondyloarthritis (SpA) or a type of psoriatic arthritis (PsA), but others maintain that these should be considered as separate diseases [11].

References: [3] - Search result 6 [4] - Search result 7 [5] - Search result 5 [6] - Search result 6 [8] - Search result 8 [11] - Search result 11 [12] - Search result 12

Treatment Options for SAPHO Syndrome

SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis) syndrome is a rare inflammatory disorder that affects the skin, joints, and bones. While there is no cure for SAPHO, various treatment options are available to manage its symptoms.

Medications Used in SAPHO Treatment

• Non-Steroidal Anti-Inflammatory Drugs (NSAIDs): These are often the first line of treatment for SAPHO syndrome, as they help reduce inflammation and pain. Examples include ibuprofen and naproxen.
• Anti-Rheumatic Drugs: Medications like colchicine, corticosteroids, and biphosphonates may be prescribed to manage symptoms.
• Disease-Modifying Agents: Infliximab, a tumor necrosis factor (TNF) blocker, has been shown to be effective in treating SAPHO syndrome. Other disease-modifying agents like etanercept and adalimumab may also be used.
• Bisphosphonates: These medications can help reduce bone turnover and prevent further damage.

Other Treatment Options

• Corticosteroids: These may be prescribed to manage inflammation and pain.
• Sulfasalazine: This medication is sometimes used to treat SAPHO syndrome, particularly in cases where NSAIDs are not effective.
• Methotrexate: This disease-modifying agent may be used in more severe cases of SAPHO syndrome.

Management by Specialists

• Rheumatologists: These specialists often manage the joint symptoms associated with SAPHO syndrome and prescribe medications to treat them.
• Dermatologists: Skin specialists may be involved in treating the skin manifestations of SAPHO syndrome, such as acne and pustulosis.

It's essential to note that treatment for SAPHO syndrome is typically symptomatic, meaning it focuses on managing symptoms rather than curing the underlying condition. A multidisciplinary approach involving various healthcare professionals may be necessary to effectively manage this complex disorder.

References:

• [1] by W Cheng · 2022 · Cited by 41 — Infliximab is an anti-TNF commonly used in SAPHO treatment.
• [3] by SWS Li · 2023 · Cited by 6 — Treatments reported in the literature include antimicrobial agents, non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, bisphosphonates, ...
• [8] by C Matzaroglou · 2009 · Cited by 87 — Treatment options for SAPHO include NSAIDs, anti-rheumatic drugs, such as colchicine, corticosteroids and biphosphonates, and disease-modifying agents, such as ...
• [10] Various medications are used in SAPHO syndrome treatment, including NSAIDs, antibiotics, steroids, sulfasalazine, methotrexate, ciclosporin, leflunomide, calcitonin, bisphosphonates, and other drugs.

Differential Diagnosis of SAPHO Syndrome

SAPHO syndrome, a rare inflammatory disorder affecting bone, joints, and skin, requires prompt recognition to avoid unnecessary procedures and long-term antibiotic therapy. The differential diagnosis for SAPHO syndrome includes various conditions that may present with similar symptoms.

Key Differential Diagnoses:

• Infectious osteomyelitis (fever and solitary lesions)
• Osteosarcoma (malignancy)
• Ewing's sarcoma (malignancy)
• Bony metastasis
• Eosinophilic granuloma
• Paget's disease
• Infectious spondylodiscitis
• Sternoclavicular osteoarthritis
• Condensing osteitis of the clavicle
• Osteonecrosis of the medial clavicular epiphysis

Important Considerations:

When considering the differential diagnosis for SAPHO syndrome, it is essential to keep in mind that infections and malignancies should be ruled out. The presence of fever and solitary lesions may indicate osteomyelitis, whereas multifocal lesions are more characteristic of SAPHO syndrome.

References:

• [5] SAPHO syndrome should be included in the differential diagnosis when evaluating patients with lytic, sclerotic, or hyperostotic bone lesions and pain.
• [4] The main differential diagnoses of SAPHO syndrome include infectious osteomyelitis, osteosarcoma, Ewing sarcoma, bony metastasis, eosinophilic granuloma, Paget's disease, infectious spondylodiscitis, sternoclavicular osteoarthritis, condensing osteitis of the clavicle, and osteonecrosis of the medial clavicular epiphysis.
• [14] When considering the differential diagnosis for SAPHO syndrome, infections and malignancies should be kept in mind. Osteomyelitis should be considered if the clinical picture includes fever and solitary lesions, unlike the multifocal lesions observed in SAPHO syndrome.