Caseous Granuloma: Tuberculosis or Chronic Recurrent Multifocal Osteomyelitis?

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Abstract

Background:

Chronic recurrent multifocal osteomyelitis (CREMO) is one of the autoinflammatory bone disorders due to disturbance in innate immune system. Up to now, there is no reported case of caseous granulomas in the CREMO. We report a boy with sterile granolumatous osteomyelitis.

Case Presentation:

A four-year-old boy presented with swelling and pain in the left wrist, malaise and bilateral erythematous pustulosis on the palmar region which had resolved spontaneously after about 7 days. The histopathology of the lesions showed severe acute and chronic inflammatory process and chronic granulomatous reaction with caseating necrosis (granulomatous osteomyelitis). The direct smear, culture and PCR for the mycobacterium tuberculosis and atypical mycobacteria were negative. About five months after initiation of the anti-mycobacterial treatment, he was referred to the rheumatology clinic with left elbow pain, effusion and decreased range of motion, and bilateral erythematous palmar pustulosis. He was diagnosed as CREMO based on two exacerbations, repeatedly negative cultures, and concomitant acute and chronic lesions in the histopathology and X-ray. Naproxen and pamidronate every 3 months were started and all other medications were stopped. Two months after the first dose of pamidronate, he became symptom-free and forearm X-ray showed disappearance of the osteolytic lesions and periosteal reactions.

Conclusion:

The diagnosis of CREMO should be considered in the patients with lytic bone lesions. In addition, the clinicians should be aware of the possibility of caseating granuloma in the cases with possible diagnosis of CREMO.

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Most cited references 22

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An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist.

Ivona Aksentijevich, Seth Masters, Polly J. Ferguson … (2009)

Autoinflammatory diseases manifest inflammation without evidence of infection, high-titer autoantibodies, or autoreactive T cells. We report a disorder caused by mutations of IL1RN, which encodes the interleukin-1-receptor antagonist, with prominent involvement of skin and bone. We studied nine children from six families who had neonatal onset of sterile multifocal osteomyelitis, periostitis, and pustulosis. Response to empirical treatment with the recombinant interleukin-1-receptor antagonist anakinra in the first patient prompted us to test for the presence of mutations and changes in proteins and their function in interleukin-1-pathway genes including IL1RN. We identified homozygous mutations of IL1RN in nine affected children, from one family from Newfoundland, Canada, three families from The Netherlands, and one consanguineous family from Lebanon. A nonconsanguineous patient from Puerto Rico was homozygous for a genomic deletion that includes IL1RN and five other interleukin-1-family members. At least three of the mutations are founder mutations; heterozygous carriers were asymptomatic, with no cytokine abnormalities in vitro. The IL1RN mutations resulted in a truncated protein that is not secreted, thereby rendering cells hyperresponsive to interleukin-1beta stimulation. Patients treated with anakinra responded rapidly. We propose the term deficiency of the interleukin-1-receptor antagonist, or DIRA, to denote this autosomal recessive autoinflammatory disease caused by mutations affecting IL1RN. The absence of interleukin-1-receptor antagonist allows unopposed action of interleukin-1, resulting in life-threatening systemic inflammation with skin and bone involvement. (ClinicalTrials.gov number, NCT00059748.) 2009 Massachusetts Medical Society

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Homozygous mutations in LPIN2 are responsible for the syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia (Majeed syndrome).

A Munnich, A Tullio-Pelet, Enrique L M Ochoa … (2005)

Majeed syndrome is an autosomal recessive, autoinflammatory disorder characterised by chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia. The objectives of this study were to map, identify, and characterise the Majeed syndrome causal gene and to speculate on its function and role in skin and bone inflammation. Six individuals with Majeed syndrome from two unrelated families were identified for this study. Homozygosity mapping and parametric linkage analysis were employed for the localisation of the gene responsible for Majeed syndrome. Direct sequencing was utilised for the identification of mutations within the genes contained in the region of linkage. Expression studies and in silico characterisation of the identified causal gene and its protein were carried out. The phenotype of Majeed syndrome includes inflammation of the bone and skin, recurrent fevers, and dyserythropoietic anaemia. The clinical picture of the six affected individuals is briefly reviewed. The gene was mapped to a 5.5 cM interval (1.8 Mb) on chromosome 18p. Examination of genes in this interval led to the identification of homozygous mutations in LPIN2 in affected individuals from the two families. LPIN2 was found to be expressed in almost all tissues. The function of LPIN2 and its role in inflammation remains unknown. We conclude that homozygous mutations in LPIN2 result in Majeed syndrome. Understanding the aberrant immune response in this condition will shed light on the aetiology of other inflammatory disorders of multifactorial aetiology including isolated chronic recurrent multifocal osteomyelitis, Sweet syndrome, and psoriasis.

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Chronic nonbacterial osteomyelitis in childhood: prospective follow-up during the first year of anti-inflammatory treatment

Christine Beck, Henner Morbach, Meinrad Beer … (2010)

Introduction Chronic nonbacterial osteomyelitis (CNO) is an inflammatory disorder of unknown etiology. In children and adolescents CNO predominantly affects the metaphyses of the long bones, but lesions can occur at any site of the skeleton. Prospectively followed cohorts using a standardized protocol in diagnosis and treatment have rarely been reported. Methods Thirty-seven children diagnosed with CNO were treated with naproxen continuously for the first 6 months. If assessment at that time revealed progressive disease or no further improvement, sulfasalazine and short-term corticosteroids were added. The aims of our short-term follow-up study were to describe treatment response in detail and to identify potential risk factors for an unfavorable outcome. Results Naproxen treatment was highly effective in general, inducing a symptom-free status in 43% of our patients after 6 months. However, four nonsteroidal anti-inflammatory drug (NSAID) partial-responders were additionally treated with sulfasalazine and short-term corticosteroids. The total number of clinical detectable lesions was significantly reduced. Mean disease activity estimated by the patient/physician and the physical aspect of health-related quality of life including functional ability (global assessment/childhood health assessment questionnaire and childhood health assessment questionnaire) and pain improved significantly. Forty-one percent of our patients showed radiological relapses, but 67% of them were clinically silent. Conclusions Most children show a favorable clinical course in the first year of anti-inflammatory treatment with NSAIDs. Relapses and new radiological lesions can occur at any time and at any site in the skeleton but may not be clinically symptomatic. Whole-body magnetic resonance imaging proved to be very sensitive for initial and follow-up diagnostics.

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Author and article information

Journal

Journal ID (nlm-ta): Iran J Pediatr

Journal ID (iso-abbrev): Iran J Pediatr

Journal ID (publisher-id): IJPD

Journal ID (pmc): IJPD

Title: Iranian Journal of Pediatrics

Publisher: Tehran University of Medical Sciences

ISSN (Print): 2008-2142

ISSN (Electronic): 2008-2150

Publication date (Print): December 2014

Publication date (Electronic): 05 December 2014

Volume: 24

Issue: 6

Pages: 770-774

Affiliations

[1 ]Children’s Medical Center, Pediatrics Center of Excellence,

[2 ]Pediatric Rheumatology Research Group, Rheumatology Research Center,

[3 ]Department of Pediatrics,

[4 ]Department of Infectious Diseases,

[5 ]Department of Pathology, Tehran University of Medical Sciences, Tehran, Iran

Author notes

[* ] Corresponding Author; Address: Division of Pediatric Rheumatology, Children’s Medical Center, No. 62 Dr. Gharib St., Keshavarz Blvd, Tehran, E-mail: ziaee@ 123456tums.ac.ir

Article

Publisher ID: ijp-24-770

PMC ID: 4442841

SO-VID: 7ac100b6-2c67-4f62-80e9-8967e844d368

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0), which allows users to read, copy, distribute and make derivative works for non-commercial purposes from the material, as long as the author of the original work is cited properly.

Caseous Granuloma: Tuberculosis or Chronic Recurrent Multifocal Osteomyelitis?

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Abstract

Background:

Case Presentation:

Conclusion:

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Point-Of-Care Testing for Tuberculosis

Most cited references 22

An autoinflammatory disease with deficiency of the interleukin-1-receptor antagonist.

Homozygous mutations in LPIN2 are responsible for the syndrome of chronic recurrent multifocal osteomyelitis and congenital dyserythropoietic anaemia (Majeed syndrome).

Chronic nonbacterial osteomyelitis in childhood: prospective follow-up during the first year of anti-inflammatory treatment

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