Diffuse Skeletal Hyperostosis Research Articles

Prevalence of ossification of the posterior longitudinal ligament (OPLL) in the Pacific populations in Auckland, New Zealand: A retrospective multicentre study.

Primary objective was to investigate the prevalence of ossification of the posterior longitudinal ligament (OPLL) in a mixed demographic region, especially in the Pacific Island population. Secondary objective was to investigate the prevalence of diabetes mellitus and cervical diffuse skeletal hyperostosis (DISH) in patients with and without OPLL. Using the local picture archiving and communication system (PACS), cervical spine computed tomography (CT) examinations over a 2-month period were retrospectively assessed for the presence of OPLL. Basic demographic data were recorded-gender, age, ethnicity, presence of cervical DISH and the presence or absence of diabetes mellitus. A total of 1692 CT examinations were included in the study. The distribution of the ethnic groups was 57.3% European, 12.09% Pacific peoples, 11.9% Māori, 11.53% Asian, 0.95% Middle Eastern/Latin American/African and 6.3% not specified. Overall, 47 cases of OPPL were identified (2.78%). The prevalence of OPPL in the Pacific ethnic groups was significantly higher than the European ethnic group 8.4% versus 0.6%, P < 0.05. The prevalence of OPLL was also significantly higher in the Asian (6.9%) and Māori (3.6%) than in the European ethnic group, P < 0.05. A significantly higher proportion of the patients with OPLL had underlying diabetes 20/47 (42.6%) compared with the study population 196/1692 (11.6%), P < 0.05. Seven cases of OPPL (14.9%) had associated cervical DISH, which was significantly higher compared with the study group (23/1692), P < 0.05. Using the Japanese Ministry of Health and Welfare classification system4, segmental type was the most common (34/47, 72.3%), followed by mixed (14.9%) and continuous types (12.8%). The prevalence of OPLL is significantly higher among the Pacific populations in Auckland. There is also increased prevalence in the Asian and Māori populations.

Not so Mutually Exclusive Diseases: A Case of Co-occurrence of Inflammatory Spondyloarthritis and Diffuse Skeletal Hyperostosis in a Young Patient.

Although the most used criteria for diffuse idiopathic skeletal hyperostosis (DISH) were designed to exclude radiographic signs of spondyloarthritis (SpA), both conditions can be present simultaneously.There are only few case reports in the literature that demonstrate the association of the two diseases.Overlap and misperception of SpA and DISH could result in undertreatment of individual patients and have a negative impact on prognosis.

RHEUMATOLOGICAL MANIFESTATION IN TYPE 2 DIABETES MELLITUS

Rheumatological manifestations of Diabetes Mellitus may be classified in: non articular, articular and bone conditions. Among non articular conditions, diabetic cheiroarthropathy, frequent in type I diabetes, the most important disorder related to limited joint mobility, results in stiff skin and joint contractures. Adhesive capsulitis of the shoulder, flexor tenosynovitis, and Duputryens and Peyronies diseases are also linked to limited joint mobility. Diffuse skeletal hyperostosis, due to calcification at entheses, is frequent and early, particularly in type 2 diabetes. Neuropathies cause some non articular conditions, mainly neuropathic arthritis, a destructive bone and joint condition more common in type I diabetes. Algodistrophy, shoulder-hand and entrapment syndromes are also frequent. Mononeuropathy causes diabetic amyotrophy, characterised by painless muscle weakness. Among muscle conditions, diabetic muscle infarction is a rare, sometimes severe, condition. Among articular conditions, osteoarthritis is frequent and early in diabetes, in which also chondrocalcinosis and gout occur. Rheumatoid arthritis (RA) and diabetes I have a common genetic background and the presence of diabetes gives to RA an unfavourable prognosis. Among bone conditions, osteopenia and osteoporosis may occur early in type 1 diabetes. Contrarily, in type 2 diabetes, bone mineral density is similar or, sometimes, higher than in non diabetic subjects, probably due to hyperinsulinemia.

Extensive Peculiar Cutaneous Form of Neurofibromatosis Type I as a New Mutation - a Case Report

Abstract Neurofibromatosis-1 (NF1) is one of the most common hereditary multisystemic disorders. The disease manifests a variety of characteristic features that include: hyperpigmentary abnormalities of the skin (café-au-lait macules, freckles in the axillae, and iris Lisch nodules) and growth of benign peripheral nerve sheath tumors (neurofibromas) in the skin. Associated extracutaneous clinical features include: skeletal abnormalities, neurological, cardiovascular, endocrine and other malformations. NF1 is caused by mutation in the neurofibromatosis-1 gene, which codes for the protein neurofibromin. The inheritance of NF1 follows an autosomal dominant trait, although about 50% of patients present with new („de novo“) mutations, and represent the first member of their family. No difference in the severity of the disease can be found in patients with familial mutations versus those with new mutations. We present a 78-year-old female patient with an extreme cutaneous form of neurofibromatosis who reported no affected family member. Apart from skin problems, she had no major health issues in childhood and adolescence, but in recent decades she had frequent headaches, occasional abdominal pain, and vision and hearing impairment. About 10 to 14 days before admission, she developed a severe cough, shortness of breath, and chest and abdominal pain. On examination, the patient of short stature (hight: 152 cm, weight: 49 kg) presented with thousands of soft nodules dispersed over the whole body, except on extensor sides of thighs and lower legs; the nodules varied in color from skin-colored, livid erythematous, to brown-grey; the nodules on the abdomen were moist, partly bleeding from the base, and accompanied by an unpleasant odor. Her feet were also densely covered by dark purple lumps, with dystrophic changes of the toe nails that were thickened, frayed, and yellowish. The skeletal abnormalities included: short stature, severe osteoporosis and osteosclerosis of the head bone structure; degenerative arthropathc-spondylotic changes of the thoracolumbar spine segment with signs of diffuse skeletal hyperostosis; pronounced degenerative changes of the lumbar spine. CT scans of the head, chest and abdomen showed the following abnormalities: flattening of the paraventricular gyri and reduction of brain parenchyma with hypodensity of the white matter in terms of cortical atrophy; periventricular bilateral small post-ischemic microvascular brain lesions of varying chronicity; in the parenchyma of the upper left lung lobe the apical presence of small areas of pleural effusion with consequent subatelectic region; distended stomach and a small inner wall herniation; hypotrophic right kidney; atherosclerotic lesions of the abdominal aorta; low grade infrarenal kinking of the abdominal aorta. Pathohistological analysis of biopsy specimen taken from the nodule corresponded with cutaneous neurofibroma. Consultative examinations of various specialists pointed to the existence of the following comorbidities: obstructive respiratory syndrome and right lobe pneumonia that were treated by antibiotics, aminophylline and dexamethasone infusions; psycho-organic syndrome without focal neurological deficit; Lisch nodules in each eye, and senile cataract. Considering the age and medical presentation of the patient, no other treatment was considered. In conclusion, this is a sporadic case of cutaneous neurofibromatosis 1 in a 78-year-old female patient who presented with extremely severe cutaneous neurofibromas, making this case at least rather peculiar.

Tumor fibroso solitario de la pleura asociado a síndromes paraneoplásicos

Solitary fibrous tumor of pleura associated with paraneoplastic syndromes Introduction: Solitary fibrous tumors of pleura (SFTP) are occasionally associated with paraneoplastic syndromes such as hypoglycemia and diffuse skeletal hyperostosis. Clinical case: We report a 60 years old male presenting with episodes of disorientation and inappropriate behavior, along with cough and weight loss. On physical examination there was digital clubbing. A chest X ray showed a big tumor in the right he- mithorax. Fasting blood glucose was 24 mg/dl and erythrocyte sedimentation rate was elevated. Large bone X rays showed hyperostosis. A needle biopsy of the tumor showed a SFTP. The tumor was excised and the final pathological diagnosis was a benign SFTP. Hypoglycemia subsided after surgery. After four months of follow up, finger clubbing was disappearing.

Kamptokormie - eine wenig bekannte Differenzialdiagnose von Hyperkyphose und ankylosierender Spondylitis

An 83-year-old patient with Parkinson's disease was referred because of pain in the thoracolumbar spine, increasing kyphosis and gait disturbance. Clinically, the main anomaly was a marked hyperkyphosis of the spine during standing and sitting which regressed while recumbent. Radiologically, spondylosis, osteochondrosis, and facet joint arthrosis demonstrated marked degeneration of the spine (diffuse skeletal hyperostosis, DISH). But the postural disorder could not adequately be explained by these pathological changes. The sacroiliac joints were age-appropriate, syndesmophytes or ankylosis typically of AS were not detectable. A diagnosis of camptocormia in connection with the known Parkinson's disease was made together with the neurologist. Intensive physio- and balneotherapy, the administration of non-steroidal anti-inflammatory drugs (NSAIDs) and an intensification of the Parkinson medication led to a slight improvement of gait disturbance and pain, but not of the tendency to hyperkyphosis. In the differential diagnosis of postural disorders in spinal diseases, especially in case of hyperkyphosis, camptocormia is of importance as a rare manifestation of different diseases, such as Parkinson's disease. The treatment of camptocormia is difficult and usually not satisfactory.

Rheumatological Manifestations in Diabetes Mellitus

Rheumatological manifestations of Diabetes Mellitus may be classified in: non articular, articular and bone conditions. Among non articular conditions, diabetic cheiroarthropathy, frequent in type I diabetes, the most important disorder related to limited joint mobility, results in stiff skin and joint contractures. Adhesive capsulitis of the shoulder, flexor tenosynovitis, and Duputryen's and Peyronie's diseases are also linked to limited joint mobility. Diffuse skeletal hyperostosis, due to calcification at entheses, is frequent and early, particularly in type 2 diabetes. Neuropathies cause some non articular conditions, mainly neuropathic arthritis, a destructive bone and joint condition more common in type I diabetes. Algodistrophy, shoulder-hand and entrapment syndromes are also frequent. Mononeuropathy causes diabetic amyotrophy, characterised by painless muscle weakness. Among muscle conditions, diabetic muscle infarction is a rare, sometimes severe, condition. Among articular conditions, osteoarthritis is frequent and early in diabetes, in which also chondrocalcinosis and gout occur. Rheumatoid arthritis (RA) and diabetes I have a common genetic background and the presence of diabetes gives to RA an unfavourable prognosis. Among bone conditions, osteopenia and osteoporosis may occur early in type 1 diabetes. Contrarily, in type 2 diabetes, bone mineral density is similar or, sometimes, higher than in non diabetic subjects, probably due to hyperinsulinemia.

HO prevention and the combined therapeutic protocol—do we really need it?

We would like to comment on the article by E.E. Pakos et al. entitled Prevention of heterotopic ossification in high-risk patients with total hip arthroplasty: the experience of a combined therapeutic protocol [5]. First, we would like to congratulate the authors for their contribution to the relevant literature. In the authors’ study, ankylosing spondylitis, hypertrophic osteoarthritis, diffuse skeletal hyperostosis, biochemical factors, male sex and a previous history of heterotopic ossication are defined as risk factors [5]. They also stated that this kind of study (combined radiotherapy and NSAID) has not been done before. They could not find a difference regarding the types of prosthesis, but found a statistically significant incidence of HO in females and in patients of osteoarthritis secondary to congenital hip dysplasia [5]. Heterotopic ossification (HO) is a common problem, especially after total hip replacement (THR) or after fracture treatment around the hip. With a brief literature review, we found studies taking an opposing view to this study in which additional medication to radiotherapy caused no improvement of the clinical outcome [1]. Combined treatments were used post actebular fractures and were found to be effective [4]. The authors stated that male sex is a risk factor, but have found HO to be increased in females. At the end of their study, they did not provide an explanation for this phenomenon. In many studies, HO was found to be more frequent in male and elderly individuals, as well as in patients with primary osteoarthritis [6]. Also in other studies, a high body mass index, low preoperative range of motion, length of operative time and large osteophytes were defined as high risk factors [3]. This could be the reason why HO is more frequent in females in this study (DDH is more frequent and the operative time is lenghtened in these patients) [5]. The author’s study is extremely valuable, but if they had a control group consisting of patients with isolated radiotherapy or an indomethacin group, then the comparison and distinction would be much easier. In some studies, even a single dose application of indomethacin was found to be effective in high-risk patients [2]. Also, this is the cheapest method of prophylaxis [2]. In conclusion, regarding the prosthetic treatment of hip fractures or osteoarthritis, HO risk factors should be clearly identified to decrease morbidity with low costs of prophylaxis. In our clinic, we use the C-reactive protein levels as a postoperative predictor of HO. We also use indomethacine because of its low cost and effectivity, regardless of the HO risk group, with convenience both for HO prophylaxis and for pain control in the postoperative setting.

Fracture of the spine in patients with ankylosis due to diffuse skeletal hyperostosis: clinical and imaging findings.

Only 12 patients with a fracture through a portion of the spine ankylosed by diffuse idiopathic skeletal hyperostosis have been reported. The purpose of this study was to determine the types of causative trauma, spinal sites at risk for fracture, complications mortality, diagnostic difficulties, and abnormalities identified only with special imaging studies in a group of 15 patients with this complication seen at our institution. The study included 15 patients with diffuse idiopathic skeletal hyperostosis who had a spinal fracture through an area of ankylosed spine. The criteria for diffuse idiopathic skeletal hyperostosis include flowing calcification or ossification along the anterolateral margin of at least four contiguous vertebral bodies; preservation of disk height in the involved areas; and absence of bony ankylosis of the apophyseal joint and erosion, sclerosis, or bony fusion of the sacroiliac joints. All spinal radiographs, tomograms, CT scans, and MR images obtained in these patients were reviewed to determine diagnostic difficulties, site and level of fracture, displacement of fractures, and extent of anatomic injury. The hospital charts of all patients were reviewed for history, physical examination, clinical status, treatment, and outcome. Fourteen fractures of the cervical spine, one of the thoracic spine, and one of the lumbar spine occurred in 15 patients. Five fractures were caused by high-energy trauma and 11 fractures by low-energy, seemingly trivial injuries. The spinal fracture caused complete quadriplegia in seven patients, incomplete quadriplegia in one patient, complete paraplegia in two patients, a central cord syndrome in two patients, and no neurologic deficit in three patients. In two of three patients in whom fracture diagnosis was delayed, paraplegia developed during the delay. Three patients died within 1 week and three more died within 6 months after injury. CT and conventional tomography enabled diagnosis of posterior element fractures not seen on plain radiographs in five patients. All fractures healed, except those in the three patients who died within a week of injury. Trivial trauma was the most common cause of fracture in the spine ankylosed by diffuse idiopathic skeletal hyperostosis. The severity of spinal cord injury in our patients was greater than in previous reports. We suggest that this may be a function of the relatively long segments of ankylosed spine (average, 16 vertebrae) in our patients providing a long lever arm for any traumatic force to act on. Patients with shorter ankylosed segments had less severe cord injuries. Delay in diagnosis of a fracture through an area of ankylosed spine associated with diffuse idiopathic skeletal hyperostosis was common if no neurologic deficit was present, and led to permanent paraplegia in two of three patients. CT and MR studies were useful in determining the anatomic abnormalities present, but were performed only in a limited number of patients.

Osteoarthritis

Large epidemiologic studies of osteoarthritis (OA) performed over the past 30 years have confirmed that OA is a ubiquitous condition, that it is linked to age, that it is more frequent and more widespread in women older than 45 years of age, and that mechanical overuse of the joints is probably instrumental in the occurrence or the location of certain cases of OA. Epidemiologic evidence points to the existence of an entity of "generalized OA" composed of three or more locations with involvement of the interphalangeals. Heredity in cases associated with distal interphalangeal OA and inflammation in cases with proximal interphalangeal OA are the factors found to be most closely correlated to generalized OA. Surveys of several series of OA of the hip have pointed to the existence of several clinicoradiologic subsets that could have different clinical correlates and various pathophysiologic mechanisms. Because several conditions, such as crystal arthropathy, diffuse skeletal hyperostosis, and subsets of OA, have been described in recent years, a reappraisal of an epidemiologic approach of OA is advisable.

Diffuse Idiopathic Skeletal Hyperostosis (Dish) and Spondylosis Deformans as Predictors of Cardiovascular Diseases and Cancer

The associations between DISH (diffuse skeletal Hyperostosis) and spondylosis deformans on the one hand and cardiovascular disease and cancer on the other were studied in a follow-up investigation of 6 167 persons in Finland. Mean duration of follow-up investigation of 6 167 persons in Finland. Mean duration of follow-up was 6.3 years. Similar methods were employed in the baseline and follow-up examination. A finding of DISH or of spondylosis was based on a reading of lateral X-ray films. Age-adjusted incidence of hypertension, ECG findings suggesting coronary heart disease (CHD), CHD history, enlargement of the heart determined by interpretation of photofluorograms, digitalis medication, cerebrovascular incidents and a history of cancer were compared in persons with and without DISH/spondylosis and free of these conditions at baseline. A further analysis, in which the data were analysed separately for DISH and spondylosis present at baseline and cases which developed during follow-up, was also carried out. A slight association between DISH and spondylosis, and hypertension probably due to obesity was observed. According to several criteria degenerative heart disease (CHD, heart enlargement, digitalis use) was associated with DISH and spondylosis, particularly in men. An association with heart enlargement was observed with DISH but not with spondylosis. No associations with cerebrovascular accidents or cancer were observed, but the paucity of cases prevents definite conclusions. Thus, there were some difference between spondylosis and DISH with regard to risk of future cardiovascular disease.

Spondylosis deformans and diffuse idiopathic skeletal hyperostosis (DISH) in Finland.

A population study with 6-year follow-up of 6 167 persons aged over 30 was carried out in nine population groups in Southern Finland. Estimation of spondylosis and DISH (Diffuse Skeletal Hyperostosis) was made from lateral chest X-rays. Reliability coefficients (kappa) in the repeat reading of 1 025 films ranged between 0.60 and 0.76. 214 cases of newly developed DISH and 1 080 of spondylosis were observed. With the exception of 4 new cases, all cases of DISH had developed in persons who had had spondylosis at baseline or developed it during the follow-up. The sexual incidence of spondylosis was fairly similar, i.e. 4 cases per 100 person years in both. Prevalence and incidence of spondylosis were highest in rural areas, in persons with strenuous occupations and in the obese. Incidence of DISH was 0.7 cases per 100 person years in men and 0.4 in women. DISH was equally common in all types of population. It was not associated with arduousness of occupation. Obesity and-to a lesser degree-diabetes mellitus and glucose intolerance were associated with DISH. Neither condition was associated with elevated serum calcium, serum cholesterol or bacteriuria. The study supports the concept that DISH is epidemiologically and pathogenetically different from spondylosis deformans.