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Objective: To determine whether an association exists between multiple myeloma and diffuse idiopathic skeletal hyperostosis (DISH).Design and Patients: Radiologic studies were performed over a 26-month period in a series of 97 consecutive patients with multiple myeloma (56 male and 41 female, aged 42-91 years).Results: Both myelomatous bone lesions and hyperostosis similar to DISH were found in these patients. The prevalence of DISH in association with multiple myeloma (21 male and 8 females patients) was higher (29.8%) than in our control group (973 patients, 449 male and 524 female) or in the general population (15-20%). The involved segments of the column were thoracic in 11 males and 7 females, cervical in 8 males and 2 females, and lumbar in 5 males and 4 females. Ossifying enthesopathy in the pelvis ("whiskering") was observed in 7 males and 1 female.Conclusions: The pathogenesis of hyperostosis remains unknown. It is possible that the coexistence of DISH and multiple myeloma is merely an association. For this reason, it is important for the real prevalence of DISH in the general population to be defined. [ABSTRACT FROM AUTHOR]

In 12 patients affected by thalassemia major who received an intensive transfusion regimen combined with continuous iron chelation therapy (desferrioxamine 50-80 mg/kg daily), radiologic abnormalities of the long bones were observed similar to those observed in rickets and scurvy. These abnormalities were associated with a growth retardation. The pathogenesis of these lesions is uncertain, but probably the toxic effect of desferrioxamine plays an important role in their development. A relative deficiency of vitamins D and/or C cannot be entirely excluded. [ABSTRACT FROM AUTHOR]

Nine patients undergoing regular dialytic treatment for more than 60 months showed clinical and radiologic features of a noninfective and destructive spondyloarthropathy. The cervical spine was most affected (100%), followed by the dorsal (three patients, 33.3%) and the lumbar spine (two patients, 22.2%). Typically, radiographs and CT scans revealed narrowing of intervertebral spaces, with destruction or sclerosis of the subchondral bone of the vertebral plate. Autopsy was performed on three patients; histologic study demonstrated the presence of large amyloid deposits containing beta 2-microglobulin (beta 2-m) in the discs and peridiscal ligaments. A radiographic follow-up of the cervical spine was performed in seven patients after a period of 12 months and showed that the bone destruction in DSA is very rapid and progressive. The lower biocompatibility of the cuprophan membranes of dialyzers is probably the factor most responsible for hyperproduction of beta 2-m and subsequently osteoarticular deposition of a new type of amyloidosis. [ABSTRACT FROM AUTHOR]

The skull in homozygous beta-thalassaemia may present several abnormalities, such as osteopenia, widening of the diploic space, and a "hair-on-end" appearance. In some cases it presents also a particular stratified appearance caused by a variable number of osseous lamellae, parallel with the inner table. This "lamellated skull" was observed in 16 out of 150 patients affected by the disease (10.6%). Possible mechanisms are discussed. The lamellar osseous changes could be due to repeated periosteal osteoblastic reactions to the sinusoidal neovascularization associated with marrow hyperplasia in poorly transfused patients. [ABSTRACT FROM AUTHOR]

The main lesions of the skull and hand, observed in a group of hypertransfused beta-thalassaemic patients, are compared with a control group of low-transfused patients. Bony abnormalities reflect the relationship between proliferating bone marrow and bone cortex, and hypertransfusion therapy will prevent development of lesions only if established early in life. If this is done, the diploë in the skull may become normal, overgrowth of facial bone is moderate, pneumatisation of the paranasal sinuses is not completely prevented, and the "hair-brush" pattern may disappear completely. A normal appearance of the hand in adequately treated patients differentiates between prepubertal patients and adults. [ABSTRACT FROM AUTHOR]

Xeroradiographic investigations of the skull, hand, and elbow were performed on 27 patients with homozygous beta-thalassaemia. The results were compared with plain radiographic examinations. Xeroradiography, because of its technical properties (i.e. edge contrast enhancement and wide latitude), was shown to demonstrate cortical thinning of long bones, swelling of the diploic space in the skull, and reticulated patterns in the elbow better than standard radiography. Moreover, the use of "positive" mode imaging was shown to have advantages in the study of the skull and extremities. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the involvement of the bursa located next to the head of the 5th metatarsal bone in patients with psoriatic arthritis (PsA) in comparison with the other seronegative spondyloarthritis (SpA)., Methods: All patients with PsA seen during a period of 24 months were enrolled. The control group included healthy subjects and patients with the other SpA. All subjects underwent clinical and ultrasound (US) examination of the lateral surface of the 5th metatarsal., Results: 150 PsA patients (88 M; 62 F), 172 SpA (107 M; 65 F), and 95 healthy controls (58 M; 37 F) were evaluated. Based on clinical and US evaluation, bursitis was diagnosed in 17/150 (11.3%) PsA patients but in none of the SpA (P < 0.0001) and healthy (P = 0.0002) controls. In detecting bursitis, US was more sensitive than clinical examination, although the difference did not reach statistical significance (P = 0.09)., Conclusion: The bursa of the 5th metatarsophalangeal joint appears to be involved in PsA more frequently than by chance. If confirmed by other studies, this finding could be considered as a distinctive clinical sign of PsA, useful for differential diagnosis with the other SpA. In asymptomatic patients, US proved to be more sensitive in the detection of bursitis.

Objectives: Adult-onset Still's disease (AOSD) is a potentially crippling or life-threatening rare disease that may be self-limited, intermittent, and chronic. Clinical predictors of outcome are still lacking, as is information on the rate of progress of its chronic course. The main objective is to identify factors that improve our ability to predict the course of AOSD, and factors associated with the rate of progress of its chronic course. A comparison with the literature is included., Methods: A retrospective cohort observational study conducted at the tertiary-referral Rheumatology Unit in Ferrara, Italy., Results: Seventy-six patients (44 females and 32 males) referred to the Unit and who satisfied the criteria for AOSD were identified. Our findings on white AOSD patients are largely compatible with those previously published. Ferritin level, as well disease activity score (DAS(28)), is associated with the rate of progression of the articular manifestations of the disease. A polyarthritis persisting over 6 months is associated with the development of a chronic articular course, irrespective of the size of the involved joints., Conclusions: Ferritin, being associated with the course of AOSD, could play a role in the diagnosis of the disease. Together with DAS(28), it might also serve as a useful predictor for the rate of progress of the chronic course of the disease, as measured with simple erosion narrowing score., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: To assess the basic features and outcomes of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome., Methods: We identified all patients seen in our unit between 1990 and 2008 diagnosed according to the proposed inclusion criteria with SAPHO syndrome, who had a followup of at least 2 years., Results: Seventy-one patients (48 women, 23 men) with SAPHO syndrome were identified. The median disease duration at the end of followup was 10 years (interquartile range [IQR] 7-15 years), and the median followup duration was 11 years (IQR 6-11.5 years). Six patients were diagnosed with Crohn's disease. Fourteen patients had never had cutaneous involvement, but 8 patients presented >1 skin manifestation. Nine patients (13%) presented a limited (<6 months) monophasic disease course, 25 cases (35%) had a relapsing-remitting course, and 37 patients (52%) had an acute painful phase with a prolonged course lasting >6 months. A total of 4% of the patients were HLA-B27 positive. Female sex (odds ratio [OR] 7.2, 95% confidence interval [95% CI] 2.2-22.9) and the presence at onset of anterior chest wall (ACW) involvement (OR 5.7, 95% CI 1.8-18.1), peripheral synovitis (P = 0.0036), skin involvement (OR 10.3, 95% CI 3.4-31.1), and high values of acute-phase reactants (OR 7.7, 95% CI 2.7-22) were correlated with a chronic disease course and involvement of new osteoarticular sites., Conclusion: A chronic course is the more common evolution of SAPHO syndrome. Female sex, elevated erythrocyte sedimentation rate and C-reactive protein values, ACW involvement, peripheral synovitis, and skin involvement at the onset seem to be associated with a chronic course.

Imaging represents a cornerstone for diagnosing and monitoring rheumatic diseases. In the last few years, with the availability of highly effective therapies, demand for the technical performance of imaging has increased exponentially, leading to rapid development of new technologies such as magnetic resonance imaging (MRI) and ultrasound (US). In both clinical practice and clinical trials, there is a need for tools that are sensitive to change and to therapy response, which are able to depict inflammatory changes early, before irreversible joint damage has occurred. Despite these advances, conventional radiography (CR) and bone scintigraphy (BS), the two oldest imaging tests, continue to provide enormous diagnostic and prognostic help for the study of many musculoskeletal disorders. Furthermore, CR is an inexpensive, widely available and reproducible tool for evaluating and monitoring structural damage. This chapter focuses on the roles of CR and BS in rheumatological clinical practice, taking into account their performance in comparison with the newer imaging techniques.

Transient osteoporosis of the hip and regional migratory osteoporosis are uncommon and probably underdiagnosed bone diseases characterized by pain and functional limitation mainly affecting weight-bearing joints of the lower limbs. These conditions are usually self-limiting and symptoms tend to abate within a few months without sequelae. Routine laboratory investigations are unremarkable. Middle aged men and women during the last months of pregnancy or in the immediate post-partum period are principally affected. Osteopenia with preservation of articular space and transitory edema of the bone marrow provided by magnetic resonance imaging are common to these two conditions, so they are also known by the term regional transitory osteoporosis. The appearance of bone marrow edema is not specific to regional transitory osteoporosis but can be observed in several diseases, i.e. trauma, reflex sympathetic dystrophy, avascular osteonecrosis, infections, tumors from which it must be differentiated. The etiology of this condition is unknown. Pathogenesis is still debated in particular the relationship with reflex sympathetic dystrophy, with which regional transitory osteoporosis is often identified. The purpose of the present review is to remark on the relationship between transient osteoporosis of the hip and regional migratory osteoporosis with particular attention to the bone marrow edema pattern and relative differential diagnosis.

A 39-year-old woman arrived to the emergency department complaining of a constant, progressive, left flank pain, with no beneficial effect from spasmolytic and nonsteroidal antiinflammatory drugs. Two years before, she suffered another episode of right flank pain and stranguria, but instrumental examinations (ultrasonography, urography) remained negative. Besides a mild tenderness in the left flank, physical examination was normal. Blood chemistry panel showed leukocytosis (17.2 x 10(3) mL, neutrophils 82.8%) and a slight increase of serum lactate dehydrogenase (LDH) (543 U/L versus 230 to 460 U/L). Urinanalysis showed a slight hemoglobinuria (0.5 mg/dL), and sediment contained some red cells and leukocytes. Diagnostic examinations (ultrasonography, computed tomography) showed a left renal nonhomogeneous space-occupying lesion, orientative for renal malignancy. She was transferred to the urology department and operated. Both intraoperatory and histological diagnosis was ischemic infarction and, after exclusion of all possible underlying causes, final diagnosis was idiopathic renal infarction. Diagnostic procedures and literature reports are discussed.

Objective: Rheumatoid arthritis (RA) is an autoimmune disorder of unknown etiology characterized by symmetric, erosive synovitis and sometimes multisystem involvement. It affects 1% of the adult population and exhibits a chronic fluctuating course which may result in progressive joint destruction, deformity, disability and premature death. We review the literature data relative to the peculiar pathologic features of the disease shown by diagnostic imaging techniques., Methods: All our patients were classified according to the diagnostic criteria of the American Rheumatism Association (1987). Plain radiography remains the diagnostic technique of choice, but ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI) are also used., Results: Clinically articular involvement presents as pain, swelling, stiffness and motion impairment. The patients with positive rheumatoid factor are > 70% likely to develop joint damage or erosions within 2 years of disease onset. Any joint can be involved, but the proximal interphalangeal and metacarpophalangeal joints of the hand and the wrist are preferential sites, as well as the metatarsophalangeal joint of the foot, the knee and the joints of the shoulder, the ankle and the hip. Symmetry is the hallmark of joint involvement. The synovium of bursae and tendon sheaths is also affected. Soft tissue (subcutaneous nodules), muscles (weakness and atrophy) and vessels (vasculitis) may also be involved. Systemic involvement may result in Felty's syndrome, metabolic bone disorders (i.e. osteoporosis), Sjögren syndrome and pleuropulmonary abnormalities (pleural effusion, fibrosing alveolitis, constrictive bronchiolitis). The earliest abnormalities consist in synovial proliferation, soft tissue swelling, and osteoporosis. At a slightly later stage, the inflamed synovial tissue ('pannus') extends across the cartilage surface, leading to chondral erosions and small bone erosions at the joint margin (bare areas). Marginal and central erosions follow in advanced stages and finally fibrous ankylosis, joint deformities (subluxations and dislocations), fractures and fragmentations are typical findings of more advanced RA., Conclusion: RA is a frequent joint disorder with a characteristic radiographic picture. Joint involvement patterns are sufficiently common to permit accurate diagnosis, especially when fusiform soft tissue swelling, regional osteoporosis, marginal and central erosions and diffuse loss of interosseous space are present. Conventional radiography remains the standard imaging technique for joint studies in the patients with suspected RA. US is recommended to diagnose soft tissue involvement (joint effusion). CT is very useful for showing abnormal processes in complex joints (sacroiliac and temporomandibular joints and craniocervical junction) which are difficult to depict completely with conventional radiography. Magnetic resonance applications include the assessment of disease activity: in particular, this technique may be the only tool differentiating synovial fluid and inflammatory pannus.

A painful and rapidly progressive form of periodontitis--involving both soft tissue and bone, with gingival bleeding and loss of teeth--was observed in HIV-patients in the mid-80's. Today, there are few reports regarding the real incidence of periodontitis in HIV populations: however, it seems not as high as first supposed on discovering the disease, and bacterial plaque is moderate, compared with "conventional" periodontitis. Since there are few radiologic studies, the Authors report on the clinical-radiographic patterns of periodontitis in 20 HIV patients, compared to 20 normal controls. All the subjects were submitted to clinical-instrumental investigations (clinical tests, periodontal sampling, DMF index), and panoramic radiography. To assess periodontal disease severity, bone pocket depth is investigated radiographically, defined as the distance between the highest point of alveolar bone and root apex, at the mesial and distal aspects of all the teeth. We measured four alveolar quadrants, from the first premolar to the second molar. Statistical analysis was carried out with one way ANOVA test and non-parametric Kruskal-Willis's test; statistical significance is accepted at the probability level p < 0.05. Clinical-radiographic results demonstrated minimal bone loss and little teeth mobility, in the early stage of disease; involvement of total gingival attachment with partial bone sequestration at muco-gingival line, in the moderate stage; severe bone loss with soft tissue necrosis and risk of teeth exfoliation, in the advanced stage. Gingival tartar was also found. A significant statistical difference was demonstrated between the two examined populations. HIV-related periodontitis may represent one of the various features of the clinical picture of HIV infection, which must not be underestimated and mistaken for "conventional" adult periodontitis. If the diagnosis of periodontic disease is essentially clinic, radiography remains nevertheless important, because it yields data on bone status, integrity of lamina dura and morphology of roots, which data help make diagnosis and prognosis more reliable.