Chronic Recurrent Multifocal Osteomyelitis and Synovitis Acne Pustulosis Hyperostosis and Osteitis Syndromes

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Chronic recurrent multifocal osteomyelitis (CRMO) is a sterile inflammatory disorder that usually occurs at multiple sites, e.g., long bones, spine,

Table 6 Musculoskeletal Infections in Adolescents

Musculoskeletal complaint (cause)

Pattern of arthritis

Clinical characteristics

Gonococcal arthritis (Neisseria gonorrhoea)

Bacterial arthritis and osteomyelitis (Staphylococcus, Streptococcus, Pseudomonas aeroginosa, gram-negative infections)

Reactive arthritis (Salmonella, Shigella, Yersinia)

40% mono-arthritis 30% oligo-arthritis 30% polyarticular Asymptomatic tenosynovitis in 2/3, especially dorsum of hands and wrists

Mono-arthritis, usually lower limb

Asymmetrical lower limb oligoarthritis most common

Frequent cause of acute septic arthritis in adolescence. Arthritis occurs as part of the disseminated phase of the disease in 1-3% of patients with symptomatic infection and in up to 0.3% of asymptomatic carriers. Initial presentation is with systemic features, dermatitis, tenosynovitis, and migratory polyarthralgia or polyarthritis (bacteraemic phase). This is followed by a progressive localization of joint symptoms and effusions in one or more joints (septic joint phase).

Septic arthritis usually occurs in a single joint, most commonly the hip, knee, or ankle. Osteomyelitis should be suspected in the presence of fever and severe bone pain extending beyond joint margins, with or without local swelling or pseudoparalysis, particularly in immunocompromised patients.

Reactive arthritis secondary to enteric organisms is relatively common. The arthritis can be very painful, but is usually short-lived. The presence of HLA B27 increases the risk of the arthritis being more prolonged and is positive in nearly 2/3 of adults and adolescents with reactive arthritis. Less commonly, nongonococcal urethritis can cause a reactive arthritis. In adolescents, there is a greater involvement in the upper limb (mainly wrist) compared to adults


Table 6 Musculoskeletal Infections in Adolescents (Continued)

Musculoskeletal complaint (cause) Pattern of arthritis

Lyme disease (Borrelia burgdorferi) Arthritis is mono-articular in 70% of patients and oligo-articular in 25%

Viral arthritis (rubella, mumps, Symmetrical polyarthritis, oligo parvovirus B19, HIV, hepatitis B arthritis or arthralgia and less commonly EBV, CMV, coxsackie B, adenovirus 7, herpes simplex, varicella zoster, and echoviruses) Small- and large-joint arthritis

Rheumatic fever group A Migratory polyarthritis of large joints streptococcal infection. Migratory arthritis, often short-lived.

Clinical characteristics

Endemic in parts of the United States, Europe, and Asia. Higher prevalence in children and adolescents (22-60%). Intermittent arthritis may appear weeks to months after infection, and may be the only clinical manifestation. Large effusions tend to occur with warmth of the joint, but little pain

Reactive arthritis can occur after viral infections or after vaccinations. Joint symptoms occur more frequently in adolescence compared to childhood following rubella and mumps infections. Small- and large-joint involvement occurs. A variety of musculoskeletal presentations are recognized in up to 75% of patients with HIV, including a mild polyarticular arthralgia, incomplete Reiter's syndrome, mono-articular or oligo-articular arthritis. Following hepatitis B infection, a disease resembling serum sickness can be seen in up to 20% adults with a polyarthritis and rash. Another 40% can get arthralgia.

Remains endemic in the developing world, with increasing incidence in the developed world. A non-erosive arthritis occurs in 47-100% of patients and is the presenting feature in 14-42%.

Brucella (small, gram-negative coccibacilli transmitted to humans via contact with infected animals or their secretions)

Mono-articular arthritis in 70% Oligo-articular in 30%

Postinfectious myositis (influenza A Myositis and B, coxsackie B virus or others)

Pyomyositis (Staphylococcus aureus, Myositis Staph, epidermidi, group A Streptococcus)

Abbreviations'. CMV, cytomegalovirus; EBV, Epstein-Barr virus. Source: Adapted from Refs. 111-113.

Rarely seen in developed countries, but remains an important cause of septic arthritis in the third world and Middle East in all age groups. Most common method of infection is through the consumption of unpasteurized goat's milk. The disease is characterized by multi-system involvement. Arthritis is the second most common manifestation after fever and is seen in 33-40% of patients. In adolescents, oligo-articular disease is seen more frequently, and the spine and small joints are usually spared.

A transient viral myositis can follow viral infections, particularly influenza or coxsackie. Other infections that can cause a myositis include toxoplasmosis, trichinosis, schistosomiasis, and trypanosomiasis

More common in the developing world. Presents initially with an insidious onset of dull, cramping pain, with or without low-grade fever. This is followed by an increased magnitude of symptoms accompanied by systemic signs (supportive phase)

and pelvic bones. It occurs mainly in children and adolescents. It is characterized by prolonged, fluctuating and recurrent episodes of pain over several years. Although histopathological and laboratory findings are nonspecific they are essential to exclude an infective or malignant process. Plain radiographs usually reveal changes consistent with a chronic osteomyelitis. Metabolically active lesions may be detected on a radioisotope bone scan and MRI may be extremely helpful (114). NSAIDs form the base of the treatment pyramid with glucocorticoids reserved for non-responders. Nonresponders to steroid therapy or those on unacceptably high doses may be treated with second line agents e.g., sulphasalazine, methotrexate or pamidronate. Pustulosis is more common in multifocal cases (115), reinforcing the concept that synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome may be part of the same disease spectrum as CRMO. Treatment is with a similar hierarchy of drugs (116).

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