An Unusual Association of Chronic Recurrent Multifocal Osteomyelitis, Pyoderma Gangrenosum, and Takayasu Arteritis

Vettiyil, George Ipe; Punnen, Anu; Kumar, Sathish

doi:10.3899/jrheum.160491

Cited by 18 publications

(11 citation statements)

References 5 publications

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“…Other reported associated diseases are pyoderma gangrenosum, ankylosing spondylitis, and juvenile idiopathic arthritis ( 92 ). Vettiyil et al reported a patient who suffered from all TA, pyoderma gangrenosum, and chronic recurrent multifocal osteomyelitis ( 93 ).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Takayasu Arteritis

2018

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Takayasu arteritis is an idiopathic granulomatous vasculitis of the aorta and its main branches and it constitutes one of the more common vasculitides in children. Inflammation and intimal proliferation lead to wall thickening, stenotic or occlusive lesions, and thrombosis, while destruction of the elastica and muscularis layers originates aneurysms and dissection. Carotid artery tenderness, claudication, ocular disturbances, central nervous system abnormalities, and weakening of pulses are the most frequent clinical features. The diagnosis is usually confirmed by the observation of large vessel wall abnormalities: stenosis, aneurysms, occlusion, and evidence of increased collateral circulation in angiography, MRA or CTA imaging. The purpose of this revision is to address the current knowledge on pathogenesis, investigations, classification, outcome measures and management, and to emphasize the need for timely diagnosis, effective therapeutic intervention, and close monitoring of this severe condition.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Takayasu Arteritis

2018

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“…The references of the retrieved articles were also reviewed for this purpose. Three case reports ( 6 , 10 , 11 ) were found between the years 1995-2017. Among these reports, one case of TAK was complicated by CRMO ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

“…Three case reports ( 6 , 10 , 11 ) were found between the years 1995-2017. Among these reports, one case of TAK was complicated by CRMO ( 6 ). The other case reports presented in Table ( 12 - 14 ) were found from the reference list of the report by Ferguson et al ( 7 ).…”

Section: Discussionmentioning

confidence: 99%

“…Although both TAK and sterile osteomyelitis have similar comorbidities, the relationship between these two conditions has been poorly investigated. Recently, Vettiyil et al described an unusual association among chronic recurrent multifocal osteomyelitis (CRMO), pyoderma gangrenosum (PG), and TAK ( 6 ). CRMO represents 2-5% of the cases of osteomyelitis and is the term that is often used in pediatrics.…”

Section: Introductionmentioning

confidence: 99%

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Takayasu Arteritis Coexisting with Sclerosing Osteomyelitis

et al. 2018

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We report a rare case of a 27-year-old woman with Takayasu arteritis (TAK) complicated by diffuse sclerosing osteomyelitis. She first presented with sclerosing osteomyelitis of the right mandible without evidence of arteritis in the carotid arteries. Eight months later, she complained of left neck pain, and imaging studies revealed the presence of arteritis in the left carotid artery. She was diagnosed with TAK, and immunosuppressive treatment was initiated, which was effective for both the arteritis and the osteomyelitis. Osteomyelitis is an important complication of TAK and bone scintigraphy is useful for its detection.

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“…As CRMO is the diagnosis of exclusion and can imitate other inflammatory bone conditions, it is thought to be underestimated [ 6 ]. Also, there are more cases emerging associated with other auto-inflammatory and autoimmune diseases, for example related to disorders with skin diseases [ 7 , 8 ], peripheral arthritis [ 9 – 11 ], concomitant inflammatory bowel disease [ 12 , 13 ], granulomatosis with polyangiitis (GPA, formerly known as Wegener granulomatosis) [ 14 , 15 ] and others [ 16 – 19 ]. An association with several auto-inflammatory conditions can be detected in about one-third of CRMO patients [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Unusual case of chronic recurrent multifocal osteomyelitis

et al. 2018

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BackgroundChronic recurrent multifocal osteomyelitis (CRMO) is a rare auto-inflammatory bone disorder that primarily affects young girls, with a mean age of 10 years at onset. Generally, it is a self-limited disease. However, recent data indicate that more than 50% of patients have a chronic persistent disease and about 20% a recurring course of this condition. Also, there are more cases reported with associated auto-inflammatory and autoimmune diseases. In this case report, we present a rare case of sporadic CRMO in which the patient eventually developed C-ANCA (cytoplasmic anti-neutrophil cytoplasmic antibodies)-associated renal vasculitis and hyperparathyroidism.Case presentationA 14 year old female patient was brought to the emergency department with a sudden onset of left leg pain and oedema. After physical evaluation and initial investigation, she was diagnosed with femoral and pelvic deep vein thrombosis. While searching for possible thrombosis causes, osteomyelitis of the left leg was identified. Additional CT and MRI scans hinted at the CRMO diagnosis. Due to the multifocal lesions of CRMO, endocrinological evaluation of calcium metabolism was done. The results showed signs of hyperparathyroidism with severe hypocalcaemia. Moreover, when kidney damage occurred and progressed, a kidney biopsy was performed, revealing a C-ANCA associated renal vasculitis. Treatment was started with cyclophosphamide and prednisolone according to the renal vasculitis management protocol. Severe metabolic disturbances and hyperparathyroidism were treated with alfacalcidol, calcium and magnesium supplements. Secondary glomerulonephritis (GN) associated hypertension was treated with ACE (angiotenzine converting enzyme) inhibitors. Anticoagulants were prescribed for deep vein thrombosis. After 1.5 years of treatment, the patient is free of complaints. All microelement and parathormone levels are within normal range. Kidney function is now normal. To date, there are no clinical or diagnostic signs of deep vein thrombosis.ConclusionsThis case report presents a complex immunodysregulatory disorder with both auto-inflammatory and autoimmune processes. We hypothesize that the long lasting active inflammation of CRMO may induce an autoimmune response and result in concomitant diseases like C-ANCA-associated vasculitis in our patient. Any potential specific pathogenic relationships between these two rare pathologies may need to be further studied. Furthermore, there is a lack of specific biomarkers for CRMO and more studies are necessary to identify CRMO’s characteristic patterns and how to best monitor disease progression.

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An Unusual Association of Chronic Recurrent Multifocal Osteomyelitis, Pyoderma Gangrenosum, and Takayasu Arteritis

Cited by 18 publications

References 5 publications

Takayasu Arteritis

Takayasu Arteritis

Takayasu Arteritis Coexisting with Sclerosing Osteomyelitis

Unusual case of chronic recurrent multifocal osteomyelitis

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