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2018 ; 16
(1
): 49
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Unusual case of chronic recurrent multifocal osteomyelitis
#MMPMID30053822
Snipaitiene A
; Sileikiene R
; Klimaite J
; Jasinskiene E
; Uktveris R
; Jankauskaite L
Pediatr Rheumatol Online J
2018[Jul]; 16
(1
): 49
PMID30053822
show ga
BACKGROUND: Chronic 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
PRESENTATION: A 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. CONCLUSIONS: This 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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