Imaging features of periostitis as a manifestation of IgA vasculitis: A case report.

INTRODUCTION: Periostitis in systemic vasculitis is very rare with only a few previously reported cases. The reported cases were seen in polyarteritis nodosa or Takayasu arteritis. We report the first case of periostitis associated with IgA vasculitis with demonstration of computed tomography (CT), magnetic resonance imaging (MRI) features, and serial changes of radiographs. PATIENT CONCERNS: A 74-year-old man visited an orthopedic outpatient clinic for pain in both lower legs and left ankle pain. He underwent a total ankle arthroplasty of the left ankle 3 years ago. His medical history disclosed IgA vasculitis/nephropathy caused by cephalosporin antibiotic class 5 months earlier. Plain radiography, MRI of the right lower leg, CT scan of the left ankle showed single lamellar to spiculated periosteal reactions at both tibia, fibula and left calcaneus. There was no evidence of bone or soft tissue mass lesions or cortical destruction. DIAGNOSIS: We concluded that this was a case of periosteal reactions associated with IgA vasculitis for the following reasons: (1) periosteal biopsy was negative for tumor. (2) there was no pulmonary lesion on chest radiography and CT, no history of trauma, inflammatory arthritis, metabolic disease, or genetic disease that could cause periostitis at multiple bones, (3) the initial MRI showed predominant signal changes around the tibial and fibular shafts, possibly explaining subsequent periosteal reactions, and (4) symptoms subsided with conservative treatment and follow-up radiographs showed remodeling of periosteal reactions. INTERVENTIONS: The patient took conservative management. OUTCOMES: His calf pain improved, and a radiograph 7 months later showed remodeling to the solid or single lamellar periosteal reaction along the both tibia and left fibula. CONCLUSION: Awareness of this uncommon manifestation would help differential diagnosis of periostitis and could help decrease the patient's anxiety. It should also be noted that periosteal reactions by benign entities could cause aggressive-looking periosteal reactions in post-operative regions.

[What's new in internal medicine?]. / Quoi de neuf en médecine interne?

Among diagnostic progress over the last three years in internal medicine, Antisynthetase Syndrome is now more easily recognised with the diffusion of laboratory tests for research of antibodies against tRNA synthetases (Anti JO1, anti PL7, Anti PL12). In two third of cases, these antibodies are found despite absence of antinuclear antibodies. Hence, we have to search them specifically in patients with polyarthritis associated with myositis, cutaneous manifestations (Raynaud phenomenom and "mechanic'hands") and interstitial lung disease. Discovery of asymptomatic mutation in the L ferritin coding sequence help us to better understand the "unexplained" hyperferritinemia. Initially described by japonese gastroenterologists, auto immune pancreatitis in fact a part of a systemic sclerosing disease with a biochemical hallmark: in crease of a subclass of immunoglobulins G (IgG4). A new pediatric disease due to a deficiency of the interleukin1 receptor antagonist (multifocal aseptic osteitis, periostitis, stomatitis, disseminated pustulosis) help us to better understand unexplained auto inflammatory diseases. The therapeutic progress is primarily due to an explosion of biological therapies, particularly four of them very useful for internists (in an off label use) : Interleukin 1 inhibitors (anakinra, Canakinumab) to treat some auto inflammatory diseases (cryopirin associated periodic syndromes and deficency of interleukin 1 receptor antagonist), monoclonal antibody against interleukin 5 (mepolizumab) to treat some hypereosinophilic syndromes and Churg and Strauss angiitis, interleukin 6 inhibitiors to treat multifocal Castleman's disease and adult Still disease, a monoclonal antibody against vascular endothelial growth factor (Bevacizumab) to treat hereditary hemorrhagic telangiectasia.

Inflammatory arthropathy in MRL hematopoietic chimeras undergoing Fas mediated graft-versus-host syndrome.

OBJECTIVE: To determine whether overexpression of the Fas ligand (FasL) on activated lpr T lymphocytes could induce arthritic lesions when grafted into syngeneic wild-type MRL mice expressing normal Fas receptor levels. METHODS: Lethally irradiated MRL+/+ mice were reconstituted with congenic MRL/lpr bone marrow cells and splenocytes overexpressing FasL. Fas-mediated cytotoxic properties of repopulating lpr splenic lymphocytes were evaluated in vitro. Simultaneously, the hind paw ankles of the hematopoietic chimeras were histologically examined. RESULTS: The lpr lymphocytes repopulating the spleen overexpressed FasL and had in vitro Fas-mediated cytotoxic activity. Simultaneously, in vivo, articular (synovitis, pannus) and periarticular (periostitis) inflammation with bone resorption were observed. CONCLUSION: Arthritic lesions may be induced in Fas-expressing recipients by persistent engrafted syngeneic lymphocytes overexpressing FasL.

The close correlation between symptoms and disease expression in HLA B27 positive individuals.

Following demonstration that 20 percent of presumed "healthy" HLA B27 positive individuals develop symptomatic ankylosing spondylitis, a controlled follow-up assessment of the remaining "asymptomatic" 80 percent was performed. The clinical and radiological study revealed that there is a close correlation between symptoms and radiologic change in HLA B27 positive subjects; those individuals remaining symptom free have normal pelvic radiographs. Ankylosing spondylitis or "asymptomatic sarcroiliitis" does not exist in a subclinical manner throughout the entire group of B27 positive subjects. Evaluation of the pelvic radiographs of both symptomatic and asymptomatic HLA B27 positive subjects and symptomatic HLA B27 negative controls demonstrated that osteitis pubis and fluffy periostitis are equally distributed among the three groups, only the frequency of sacroiliitis being statistically greater in the B27 positive symptomatic subjects.