Osteitis as a symptom of SAPHO syndrome

SAPHO syndrome is an acronym given to a spectrum of diseases with the following features: synovitis, acne, pustulosis, hyperostosis and osteitis. The main problem of the differential diagnosis in 3 cases presented in our paper was the aseptic osteitis.     

Coexistence of diffuse idiopathic skeletal hyperostosis and ankylosing spondylitis: a case report

Diffuse idiopathic skeletal hyperostosis and ankylosing spondylitis are two diseases which are listed in the differential diagnosis of each other. There have been limited numbers of case reports regarding the coexistence of both diseases in the literature. We describe a patient who demonstrated the features of diffuse idiopathic skeletal hyperostosis with coexisting features resembling ankylosing spondylitis in order to discuss the association of the two diseases. Received: 22 March 2001 / Accepted: 17 November 2001     

Ankylosing hyperostosis in American blacks: A longitudinal study

We studied 63 consecutive patients with ankylosing hyperostosis to investigate any possible difference in clinical or roentgenographic features between whites and blacks. Our data suggest that (a) the disease may not be less common among blacks, (b) presence of extraspinal hyperostosis in this disease may be more frequent in black patients, and (c) the known male preponderance of the disease among whites may not occur among blacks.     

Radiological features of long bones in synovitis, acne, pustulosis, hyperostosis, osteitis syndrome and their correlation with pathological findings

The purpose of this study was to demonstrate the radiological features of long bones in synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome and to correlate these with the clinical findings. Eleven long bone lesions in seven cases of SAPHO syndrome were examined. The patients ranged in age from 6 to 63 years, with a mean of 47 years. In all seven cases, radiography, ^99mtechnetium bone scintigraphy, CT scan, and magnetic resonance imaging (MRI) were performed. In six of the cases, bone biopsy and bone culture were carried out for 7 long bones. Seven of the involved lesions were from the shaft of the femur, one each was from the neck and the shaft of the humerus, and one was from the proximal tibia. These lesions showed radiologically hyperostosis, osteolysis, and bone infarction-like lesion. Osteolysis was occasionally accompanied by sclerotic change. Hyperostosis usually showed diaphyseal involvement, presenting low signal intensity on T₁- and T₂-weighted MR images. Histologically, these findings corresponded to massive bone necrosis, new bone formation, fibrosis, or a mixture of these associated with mild inflammatory cell infiltration. Osteolysis involved dyaphysis, metaphysis, or epiphysis associated with arthritis, and presented low signal intensity on T₁-weighted images, nonhomogeneous signal intensity lower than fat on T₂-weighted images, and high signal intensity on fat suppression images. These findings corresponded to fibrosis, granulation, and inflammatory cell infiltration with lymphocyte aggregation. Bone infarction-like lesion was observed in the shaft or neck of the femur and the humerus and accompanied by calcification and cystic change. Bone cultures were negative in all cases in which bone biopsy was performed. Although hyperostosis is thought to be a characteristic bone lesion in SAPHO syndrome, the long bone lesion can occasionally show not only hyperostosis but also osteolytsis and bone infarction-like lesions. Received: April 17, 2001 / Accepted: August 2, 2001     

Hyperostosis cranii in the elderly with various clinical symptoms

We report three elderly patients with hyperostosis cranii. Patient 1 had two episodes of unconsciousness; Patient 2, headache; and Patient 3, dementia. On the basis of Moore's classification using skull films, Patients 1 and 2 showed hyperostosis frontoparietalis and Patient 3 had hyperostosis frontalis interna. Electroencephalography showed transient generalized spike and slow wave complexes over the frontal lobes in Patient 1. Magnetic resonance images showed frontal lobes compressed by the thickness of the frontal bones in all patients and the thickness of the parietal bones in Patients 1 and 2. Since the findings in the present cases and those in the literature suggest that hyperostosis cranii could show unexpected neuropsychiatric symptoms, hyperostosis cranii should be checked in elderly patients whose presenting symptoms include epilepsy, dementia, psychiatric disease, headache and so on. Magnetic resonance images should be helpful in examining the relationship between clinical symptoms and the deformation of the brain by the skull.     

ARTHRO-OSTEITIS--A CLINICAL SPECTRUM

Arthro-osteitis is an uncommon condition which can be associatedwith palmoplantar pustulosis. It forms part of a group of conditionswhich include the synovitis, acne, pustulosis, hyperostosis,osteitis syndrome (SAPHO) and sternocostoclavic-ular hyperostosis.We report four cases illustrating the clinical spectrum of thiscondition which occurred in the absence of concomitant skinlesions. One patient had extensive aortic calcification a featurenot previously reported in this condition, which may representa low grade inflammatory aortitis. KEY WORDS: Sternocostoclavicular hyperostosis, SAPHO syndrome, Osteitis, Aortic calcification, Palmoplantar pustulosis     

Noninfectious osteitis: Part of the SAPHO syndrome

Summary SAPHO, a rare syndrome, is a recently suggested acronym for synovitis, acne, pustulosis, hyperostosis and osteitis. It encompasses many features which have been described in different but overlapping conditions. Not all of the syndrome components need to be present for inclusion in SAPHO to be justified, especially the dermatologic components. Two cases are described as examples. Clinicians should be aware of this rare disorder if positive early diagnoses are to be made in patients presenting with skeletal pain.     

IS THE HIP INVOLVED IN GENERALIZED OSTEOARTHRITIS?

Elbow pain is a common complaint and elbow hyperostosis a frequentradiological condition. However, little is known about the associationbetween the clinical and radiological findings. To evaluatethe relationship between spinal and extraspinal hyperostoticfeatures and the clinical relevance of elbow hyperostosis wehave performed the first controlled, double-blinded study of85 hospitalized probands, 33 with and 52 without thoracospinalhyperostosis on lateral chest X-ray. Elbow and shoulder hyperostosiswere graded on bilateral standard radiographs. Elbow pain wasassessed by an interviewer using a standardized questionnaireand extraskeletal causes of elbow pain were recorded. The prevalenceof elbow hyperostosis was increased in cases with thoracospinalhyperostosis compared to controls (82% versus 58%, X² = 5.32,P<0.025, n = 85, odds ratio (OR) 3.30 (95% Cl 1.16–9.35)).Similarly, the prevalence of elbow hyperostosis was increasedin cases with shoulder hyperostosis compared to controls (83%versus 60%, x² = 4.51, P<0.05, n = 84, OR = 3.20 (95% CI1.06–9.66)), emphasizing the multifocal nature of hyperostoticfeatures. Elbow pain was only slightly more prevalent in caseswith elbow hyperostosis compared to controls (21% versus l3%,x² = 0.75, NS, OR = 1.84 (95% CI 0.46–7.44)). We concludethat elbow hyperostosis is a radiological finding of doubtfulclinical relevance. KEY WORDS: Hyperostosis, Spinal osteophytosis, Elbow, Pain     

DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS (DISH) OF THE ELBOW: A CAUSE OF ELBOW PAIN? A CONTROLLED STUDY

Elbow pain is a common complaint and elbow hyperostosis a frequentradiological condition. However, little is known about the associationbetween the clinical and radiological findings. To evaluatethe relationship between spinal and extraspinal hyperostoticfeatures and the clinical relevance of elbow hyperostosis wehave performed the first controlled, double-blinded study of85 hospitalized probands, 33 with and 52 without thoracospinalhyperostosis on lateral chest X-ray. Elbow and shoulder hyperostosiswere graded on bilateral standard radiographs. Elbow pain wasassessed by an interviewer using a standardized questionnaireand extraskeletal causes of elbow pain were recorded. The prevalenceof elbow hyperostosis was increased in cases with thoracospinalhyperostosis compared to controls (82% versus 58%, X² = 5.32,P<0.025, n = 85, odds ratio (OR) 3.30 (95% Cl 1.16–9.35)).Similarly, the prevalence of elbow hyperostosis was increasedin cases with shoulder hyperostosis compared to controls (83%versus 60%, x² = 4.51, P<0.05, n = 84, OR = 3.20 (95% CI1.06–9.66)), emphasizing the multifocal nature of hyperostoticfeatures. Elbow pain was only slightly more prevalent in caseswith elbow hyperostosis compared to controls (21% versus l3%,x² = 0.75, NS, OR = 1.84 (95% CI 0.46–7.44)). We concludethat elbow hyperostosis is a radiological finding of doubtfulclinical relevance. KEY WORDS: Hyperostosis, Spinal osteophytosis, Elbow, Pain     

Diffuse idiopathic skeletal hyperostosis and new bone formation in male gouty subjects

Summary Ninety-nine males with gout were identified and their radiographs examined for features of vertebral hyperostosis and entheseal changes (diffuse idiopathic skeletal hyperostosis, DISH). Of patients over the age of 45 years 43% fulfilled criteria for diagnosis of DISH. New bone formation in other regions of the skeleton was also common. The overhanging margin sign, seen in well developed tophi, was noted only in patients with prominent new bone formation elsewhere in their skeleton. Some of the radiologic manifestations of gout may be modified by a co-existent tendency, in these patients, to form new bone. It is suggested that a common metabolic factor, possibly hyperinsulinaemia, underlies the association of gout and hyperostosis.     

Ossification of the ligamentum teres as a possible mechanism of lateral subluxation in coxopathy associated with ankylosing spinal hyperostosis.

The cases are reported of two patients with coxopathy associated with ankylosing spinal hyperostosis who showed ossification of the ligamentum teres. It is suggested that considerable ossification at the medial acetabular floor including the ligamentum teres can explain the mechanism of development of coxopathy with lateral subluxation of the femoral head which is often seen in patients with ankylosing spinal hyperostosis.     

Sternocostoclavicular hyperostosis (SCCHO) with palmoplantar pustulosis (PPP)

Three cases of sternocostoclavicular hyperostosis (SCCHO) or pustulotic arthro-osteitis (PAO) associated with plantar pustulosis are presented. Two of them were associated with psoriasis vulgaris although the pustulosis was of a different histological type in each case. Anatomo-pathological study of two of these three cases shows that this hyperostosis is secondary to an inflammatory osteomedulloperiosteal remodelling suggesting an infectious origin which remains to be proven. The relationship of this syndrome with some seronegative spondylarthropathies cannot, however, be excluded.     

Ankylosing spondylitis in nonhuman primates: the drill and the siamang.

Nonhuman primates are known to be susceptible to many of the arthritides that afflict humans. Psoriatic like spondyloarthropathies have been reported in gorillas and skeletal hyperostosis in gibbons, rhesus monkeys, and gorillas, and additional cases of both of these conditions occurring in drills (baboons) are noted in this report. One western lowland gorilla and two rhesus monkeys with clinical features consistent with ankylosing spondylitis have been documented previously. Two additional nonhuman primate species with radiographic evidence of ankylosing spondylitis are described. A siamang (gibbon) and two drills (baboons) with the classic radiographic features of ankylosing spondylitis, namely a bamboo spine and sacroiliac joint fusion, are reported.     

Das SAPHO-Syndrom: Klinisch-rheumatologische und radiologische Differenzierung und Klassifizierung eines Krankengutes von 86 Fällen

Synovitis (inflammatory arthritis), acne (pustulosa), pustulosis (psoriasis, palmoplantar pustulosis), hyperostosis (acquired), and ostitis (bland osteomyelitis) are symptoms forming the acronym SAPHO, which is a syndrome of nosologic heterogeneity. All entities forming the SAPHO syndrome are connected by a non-obligate dermatoskeletal association with an aseptic pustulous character. 86 cases were analyzed clinically, radiologically and by histology/histopathology. 31 adult patients showed the typical triad of pustulosis palmo-plantaris (psoriatica, PPP), sterno-costo-clavicular hyperostosis (SCCH), and "productive" spondylopathy, which we define as entity I. spondarthritis hyperostotica pustulopsoriatica (Spond.hyp.pp). Twelve adolescent and 13 adult patients showed entity no. II: chronic recurrent multifocal osteomyelitis (CRMO), being characterized by non-purulent osteomyelitis of plasma-cell sclerotic type, potentially being a reactive inflammatory process. 50% of the adult patients with CRMO showed PPP. Differentiation between these two entities is possible by detection of ossifying enthesiopathy in cases of Spond. hyp.pp and primarily chronic osteomyelitis in cases of CRMO. Two more entities or abortive forms of group I and II are III: the inflammatory syndrome of the anterior chest-wall (ACW syndrome) and IV: the more productive form of isolated sterno-costoclavicular hyperostosis (SCCH). Both are connected quite frequently to HLA-B-27-independent forms of spondarthritis and to pustulous dermatosis. More rarely we find osteo-articular symptoms in cases of acne pustulosa, which form group V: acne-associated spondarthritis and CRMO in the case of acne. Adult forms of CRMO with different forms of appearance (lumosacro-iliac hyperostosis with retroperitobeal fibrosis, pelvic type with affection of the hip-joint) are described. The immunologic theory of a "reactive osteomyelitis" potentially triggered by saprophytes is described. The inverse acne triad is brought in a context of skin symptoms. A case of intercurrent postpartum symptoms together with ulcerative colitis is described. Three cases of patients with Crohn's disease are described. Clinical features, radiological findings, and histopathological elements are brought together to determine the connections between the different entities and the possibilities of differentiation. With these elements together with bone-scan, it is often not necessary to obtain a bone specimen. Therapeutical possibilities, especially concerning CRMO, are discussed. "SAPHO syndrome" is more a sign-post on the way to a more subtle diagnosis when it comes to hyperostotic, skin-associated diseases, and it needs interdisciplinary work to clear the situation.     

Gerontological aspects of hyperostosis frontalis interna.

Authors discuss hyperostosis frontalis interna observed in a large number of aged persons, on the basis of age and sex distribution as well as its clinical and roentgenomorphological analysis. In various forms of the ossification of the frontal bone no significant difference was found between the localisation of hyperostosis and the clinical symptoms. On other hand, there is a direct correlation between the extension and severity of hyperostosis and the frequency of occurence of the associated symptoms (obesity, hypertension). They found the aetiological classification more adequate than the morphological categorization of Moore. Their cases are discussed 1. as partial phenomenon of the Morgagni's syndrome; 2. as independent alteration, showing no other symptoms; 3. as transitionary forms inserted between the two groups mentioned above. They discuss also the question of senile, compensatory hyperostosis frontalis interna. On the basis of the study of a large autopsy material they support the opinion that there is a direct connection of this form with old age.     

Abnormalities of the sacroiliac joints in diffuse idiopathic skeletal hyperostosis: demonstration by computed tomography

Eight patients with classical spinal radiographic features of diffuse idiopathic skeletal hyperostosis (DISH) had pelvic radiographs which suggested sacroiliac joint abnormalities. No patient had clinical features of ankylosing spondylitis. Computed tomography of the sacroiliac joints revealed several abnormalities including asymmetric intraarticular partial fusion, osteophytes with or without bridging, and vacuum phenomenon. Sacroiliac joint disease can complicate DISH.     

SAPHO综合征一例报道并文献复习

目的 探索ＳＡＰＨＯ综合征的临床特点及诊断方法。方法 报道了一例ＳＡＰＨＯ综合征并对ＳＡＰＨＯ综合征的文献进行复习。结果 本例有手足掌面脓疱疮,双侧锁骨和第一前肋骨炎,骨肥厚。骨病理切片示慢性炎症改变,故确诊为ＳＡＰＨＯ综合征。结论 ＳＡＰＨＯ综合征临床可以见到,应注意和其他疾病鉴别。     

Síndrome de sinovitis,acné, pustulosis,hiperostosis, osteítis y tratamiento con antagonistas del factor de necrosis tumoral

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis) encompasses a group of osteoarticular manifestations, especially hyperostosis in the joints and bones of the anterior chest wall and skin in the form of severe acne or hidradenitis. In the treatment of this syndrome, whose pathogenesis is unknown, many drugs have been used such as nonsteroidal anti-inflammatory agents and antibiotics. Corticosteroids, methotrexate, sulfasalazine and intravenous bisphosphonates have been used mainly in refractory cases. The development of biological treatments, indicated in certain rheumatic diseases, has provided a new therapeutic option, especially in patients without response. Anti-tumor necrosis factor-alfa agents, specifically infliximab and etanercept, have been used in isolated cases and small series. In general, the results have been satisfactory and these drugs can be an effective alternative to conventional treatments.     

Sternocostoclavicular hyperostosis: its progression and radiological features. A study of 12 cases.

Twelve cases of sternocostoclavicular hyperostosis were followed up over four to 16 years. The patients underwent repeated radiological examinations of the sternocostoclavicular joints and the sternum, and the extrasternal osseous manifestations of the disease were studied to show changes in the radiological features during long term follow up. Five of 12 (41%) patients had extrasternal manifestations. With the exception of one patient extrasternal manifestations were first detected by scintigraphs because they were asymptomatic. With respect to the sternal manifestations the initial radiological diagnosis was made during an acute phase while painful swelling over the sternum and decreased mobility of the shoulders occurred. The radiological examinations showed the signs of a proliferative destructive arthritis in most patients. In contrast with the frequent occurrences of clinical symptoms, the radiological signs of progression take several years to become detectable. There are no specific bacteriological, serological or histological findings. Usually a permanent increase in the erythrocyte sedimentation rates is found. Sternocostoclavicular hyperostosis is a slowly progressing disease, characterised by a chronic aseptic destructive sternoclavicular arthritis with a reactive low turnover sclerosis that begins in a similar way to an enthesopathy and ends after several decades with total ankylosis. The radiological identification of retrosternal proliferation of soft tissue by computed tomography was found to be a valuable criterion for the differential diagnosis from other benign hyperostotic processes of the sternoclavicular region.     

THE PREVALENCE OF PALPABLE FINGER JOINT NODULES IN DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS (DISH). A CONTROLLED STUDY

The presence of clinically palpable finger joint nodules a(Heberden'sand Bouchard's nodes) was documented in 123 consecutive caseswith diffuse idiopathic skeletal hyperostosis (DISH) of thethoracic spine and 191 matched DISH negative controls. The prevalenceof palpable finger joint nodules was almost twice as frequentin cases with spinal DISH compared to controls (46% versus 31%,X² = 7.67, ^P<0.01; multivariate adjusted odds ratio OR =1.84; 95% CI: 1.14–2.98). This increase was most markedat the proximal interphalangeal joint, in males and in patientsup to the age of 65 years. In addition and independent of othervariables such as hyperostotic features, age and sex, the prevalenceof palpable finger joint nodules was about twice as high inprobands with a history of physically heavy work compared tothose without (43% ver sus 26%, ^X = 9.18, P<0.005; multivariateadjusted odds ratio OR = 2.10; 95% CI: 1.26–3.52). Fromthese results we con clude that DISH should be considered asan independent risk factor in the development of finger jointnodules. KEY WORDS: Heberden's nodes, Diffuse idiopathic skeletal hyperostosis (DISH), Controlled study