title: Chronic Recurrent Multifocal Osteomyelitis and Thalidomide in Chronic Granulomatous Disease.
abstract: Chronic granulomatous disease (CGD) is a primary immunodeficiency that leads to severe recurrent infection and inflammatory complications that are usually difficult to diagnose and treat. Several hyperinflammation mechanisms, such as decreased neutrophil apoptosis, toll-like receptor activation imbalance, Th17 cell induction, Nrf2 activity deficiency, and inflammasome activation, have been described in CGD patients However, there have been no reports of chronic recurrent multifocal osteomyelitis as an inflammatory complication in CGD, and the differential diagnosis of this condition with infectious osteomyelitis is challenging. Thalidomide has been used to treat several inflammatory manifestations in CGD patients with good clinical results. Here, we report the case of a previously asymptomatic 11-year-old boy who consulted for difficulty walking and pain at the back of the right thigh, with increased inflammatory markers. Multifocal bone involvement was seen on bone scintigraphy, and acute-phase reactants were elevated. On the basis of a suspected diagnosis of infectious osteomyelitis, broad-spectrum antibiotic therapy was started, with no clinical response. Bone biopsy and microbiological tests yielded negative results; at that point, chronic recurrent multifocal osteomyelitis was suspected. The patient was unresponsive to nonsteroidal antiinflammatory drugs and corticosteroids. Thalidomide was started, and within 6 months, clinical and radiologic resolution of the condition was achieved with no adverse effects. More than 1 year after stopping thalidomide, the patient remained free of symptoms and inflammatory parameters are within normal levels. Thalidomide has a favorable safety profile compared with other alternatives and could be considered a feasible therapeutic option for this type of condition in selected patients.
authors.affiliation: Pediatric Infectious Diseases and Immunodeficiencies Unit, andmarti@vhebron.net.
authors.forename: Andrea
authors.initials: A
authors.lastname: Martín-Nalda
authors.affiliation: Department of Nuclear Medicine.
authors.forename: Isabel
authors.initials: I
authors.lastname: Roca
authors.affiliation: Unit of Pediatric Orthopaedic Surgery.
authors.forename: Cesar Galo
authors.initials: CG
authors.lastname: Fontecha
authors.affiliation: Department of Pharmacy.
authors.forename: Aurora
authors.initials: A
authors.lastname: Fernández-Polo
authors.affiliation: Department of Pediatric Radiology, and.
authors.forename: Ignasi
authors.initials: I
authors.lastname: Barber
authors.affiliation: Division of Immunology, Hospital Universitari Vall d'Hebron, Institut de Recerca Vall d'Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Mónica
authors.initials: M
authors.lastname: Martinez-Gallo
authors.affiliation: Pediatric Infectious Diseases and Immunodeficiencies Unit.
authors.forename: Pere
authors.initials: P
authors.lastname: Soler-Palacin
mesh_terms: D002648:Child; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D010019:Osteomyelitis / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000209:etiology; D011877:Radionuclide Imaging; D013792:Thalidomide / Q000627:therapeutic use*
chemical_list: D007166:Immunosuppressive Agents; D013792:Thalidomide