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GastroenterologyInflammatory bowel disease

 Summary 

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare disease of the bone, first described in 1972. It is a relapsing, often symmetrical and self-limiting, non-infectious condition, primarily affecting children. The association of CRMO and IBD is very rare, especially in adults.

This report describes a 37-year-old male patient with Crohn's disease who presented to hospital with recurrent bone pains. After extensive investigations he was diagnosed as having CRMO. He responded well to steroids.

 Case Report 

History

A 37-year-old Caucasian male first presented in 1992 with intermittent diarrhea and was diagnosed with Crohn's disease. In 1999 he had a pan-proctocolectomy and ileostomy. Histology of the resected specimen confirmed Crohn's disease. In February 2000 he developed pain initially in his left forearm and later in his right forearm.

Investigations and Management

X-rays showed lytic lesions in the cortex of both radii. In March 2000 he developed pain and swelling over his left thigh and right tibia. An X-ray of his left femur showed a lytic area with periosteal reaction in the medial portion of distal femoral shaft. While an X-ray of the right tibia showed a lytic area within the medulla of the mid-tibial shaft.

White cell count and C-reactive protein (CRP) were normal, but alkaline phosphatase was slightly elevated. An isotope bone scan showed increased activity in the left forearm, left and right femoral shafts, and sternum (Fig. 1(a)).

He was reviewed by the orthopedic surgeons with a provisional diagnosis of osteomyelitis, and treated with antibiotics and non-steroidal anti-inflammatory drugs (NSAIDs).

However, a magnetic resonance scan 3 months later showed altered signal within marrow cavity of mid right radius (Fig. 1(b)). Overall, bony cortex was well preserved. There was an increased signal in the mid left tibia, but the right tibia was normal. His symptoms waxed and waned, and in December 2000 a small fragment of bone discharged through the anterior aspect of his left forearm. Wound swab showed no growth.

By March 2001 his bone pains had settled, and a repeat bone scan was normal.

In December 2001 he was admitted with left thigh pain and swelling with no external sinuses. He had intermittent pyrexia. White cell count and inflammatory markers (CRP and erythrocyte sedimentation rate) were elevated. Blood cultures showed no growth.

X-rays of the left femur showed a lytic lesion in the medullary cavity with an associated periosteal reaction (Fig. 2(b)). A bone scan showed increased uptake in the mid-sternum, left tibia, left femur (Fig. 2(a)) and right elbow. An ultrasound scan and computed tomography of his abdomen and pelvis were normal.

A bone biopsy showed acute inflammatory cells and necrotic bone. A bone aspirate showed no growth, and gram stains and Ziel Nielson stains were negative. He was treated with intravenous antibiotics for 3 weeks. His symptoms, pyrexia and inflammatory markers failed to settle.

Outcome

After the diagnosis of CRMO was made he was commenced on corticosteroids. His symptoms, pyrexia and inflammatory markers dramatically settled.

 Discussion 

CRMO is an acquired disease of the skeleton that occurs predominantly during childhood and adolescence. It is a relapsing, self-limiting, often symmetrical, non-infectious condition, predominantly affecting the tubular bones of the limbs, followed by the clavicles and the spine [1, 2]. CRMO is associated with other conditions, such as palmoplantar pustulosis, psoriasis and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome [3].

The association of CRMO with IBD is very rare, and to date there have only been 10 published cases in the English literature describing an association (see Table 1).

The aetiology is unknown. It has been reported in association with other autoimmune diseases, such as Wegener's granulomatosis and Takayasu vasculitis, suggesting a possible immune response directed against bone [4]. In IBD proinflammatory cytokines released by the inflamed bowel could potentially mediate joint inflammation, bony inflammation and osteolysis [4].

It has also been postulated that CRMO is caused by a fastidious organism. Mycoplasma hominis, Propionibacterium acnes, and Coxiella burnetii have been implicated in isolated case reports. However, in general, cultures of bone biopsy specimens, blood and polymerase chain reaction techniques have been negative [5].

Plain radiographs reveal a variable pattern, such as osteolysis with a sclerotic rim, mixed lytic-sclerotic lesions, and pure sclerosis [6]. Bone scintigrams may be a more accurate investigation, as they may show clinically asymptomatic lesions and foci, which are radiographically difficult to detect [6]. MR imaging detects marrow oedema, and is a more sensitive indicator of disease.

The spectrum of histopathologic changes ranges from acute (acute inflammatory infiltration, active bone resorption and necrosis, and reactive bone formation) to subacute (predominantly lymphocytic and plasma cell infiltration) to chronic inflammation (fibroblastic organization and bony sclerosis) [7].

Diagnostic criteria for CRMO, proposed by King et al. [2], include:

	Multifocal (2 or more) bone lesions, clinically or radiologically diagnosed.
	Prolonged course (more than 6 months) characterized by varying activity of disease and with most patients being healthy between recurrent episodes of local pain, swelling and tenderness.
	Absence of response to antibiotic therapy, administered for at least 1 month.
	Typical radiographic changes of lucency surrounded by sclerosis together with increased uptake on bone scan, and.
	Lack of an identifiable organism.

A variety of therapeutic agents and operative procedures have been used in the treatment of CRMO. Antibiotics, steroids, and NSAIDs have been tried with variable success. In CRMO associated with IBD steroids, with or without other therapy of the IBD, were generally associated with improvement in the bone symptoms (Table 1).

There are no pathognomonic features and a high index of clinical suspicion is needed. CRMO should be considered in any IBD patient with unexplained bone pain, or areas of uptake on bone scan. It may be a rare extraintestinal manifestation of IBD.

Alternatively, certain individuals may be genetically predisposed to the development of both diseases. Recognizing this association is important to avoid unnecessary diagnostic procedures and to initiate appropriate therapy.

This article was first published on GastroHep.com on 22 April 2003.

 References 

1. Giedion A, Holthusen W, Masel LF, Vischer D. [Subacute and chronic ‘symmetrical' osteomyelitis]. Ann Radiol (Paris) 1972; 15 (3): 329-42. PubMed
2. King SM, Laxer RM, Manson D, Gold R. Chronic recurrent multifocal osteomyelitis: a noninfectious inflammatory process. Pediatr Infect Dis J 1987; 6 (10): 907-11.
3. Laxer RM, Shore AD, Manson D, King S, Silverman ED. Chronic recurrent multifocal osteomyelitis and psoriasis: a report of new association and review of related disorders. Semin Arthritis Rheum 1988; 17 (4): 260-70. PubMed
4. Bousvaros A, Marcon M, Treem W, Waters P, Issenman R, Couper R et al. Chronic recurrent multifocal osteomyelitis associated with chronic inflammatory bowel disease in children. Dig Dis Sci 1999; 44: 2500-7. PubMed
5. Jurriaans E, Singh NP, Finlay K, Friedman L. Imaging of chronic recurrent multifocal osteomyelitis. Radiol Clin North Am 2001; 39 (2): 305-27. PubMed
6. Demharter J, Bohndorf K, Michl W, Vogt H. Chronic recurrent multifocal osteomyelitis: a radiological and clinical investigation of five cases. Skeletal Radiol 1997; 26 (10): 579-88.
7. Chow LT, Griffith JF, Kumta SM, Leung PC. Chronic recurrent multifocal osteomyelitis: a great clinical and radiologic mimic in need of recognition by the pathologist. APMIS 1999; 107 (4): 369-79. PubMed
8. Kotilainen P, Laxen F, Manner I, Gullichsen RE, Saario RM. An aseptic inflammation of the clavicle in a patient with Crohn's disease: a potential manifestation of the SAPHO syndrome. Scand J Rheumatol 1996; 25 (2): 112-4. PubMed
9. Bognar M, Blake W, Agudelo C. Chronic recurrent multifocal osteomyelitis associated with Crohn's disease. Am J Med Sci 1988; 315 (2): 133-5.
10. Omidi CJ, Siegfried EC. Chronic recurrent multifocal osteomyelitis preceding pyoderma gangrenosum and occult ulcerative colitis in a pediatric patient. Pediatr Dermatol 1998; 15 (6): 435-8. PubMed
11. Bazrafshan A, Zanjani KS. Chronic recurrent multifocal osteomyelitis associated with ulcerative colitis: a case report. J Pediatr Surg 2000; 35 (10): 1520-2. PubMed

Table 1  Summary of cases of CRMO associated with IBD

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