SAPHO syndrome or psoriatic arthritis? A familial case study.

OBJECTIVE: To discuss the relationships between SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome and the group of spondylarthropathies. METHODS: Few reports of familial SAPHO have been published. We describe three children, two sisters and one brother, whose clinical and radiological presentation was in accordance with SAPHO syndrome. RESULTS: Two children developed psoriasis, and one child palmoplantar pustulosis. Both sacroiliac and sternoclavicular joints were involved in these three cases. Some features in our observations are also common to psoriatic arthritis. No association was found with HLA antigens, but a history of trauma preceding the onset of symptoms was present in all three children. CONCLUSIONS: We can consider that SAPHO is nosologically related to spondylarthropathies. Psoriatic arthritis could be the missing link between SAPHO and spondylarthropathies. It is likely that both genetic and environmental factors are involved.     

Haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome

OBJECTIVE—To assess haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome.
DESIGN—Retrospective observational study.
SETTING—Tertiary referral centre.
PATIENTS—94 adult patients with Eisenmenger syndrome who were diagnosed between September 1982 and July 1998.
MAIN OUTCOME MEASURES—Serum uric acid was measured in all patients, together with clinical and haemodynamic variables related to mortality.
RESULTS—Serum uric acid was raised in patients with Eisenmenger syndrome compared with age and sex matched control subjects (7.0 v 4.7 mg/dl, p < 0.0001) and increased in proportion to the severity of New York Heart Association functional class. Serum uric acid was positively correlated with mean pulmonary arterial pressure (r = 0.30, p = 0.0052) and total pulmonary resistance index (r = 0.55, p < 0.0001), and negatively correlated with cardiac index (r = −0.50, p < 0.0001). During a mean follow up period of 97 months, 38 patients died of cardiopulmonary causes. Among various clinical, echocardiographic, and laboratory variables, serum uric acid remained predictive in multivariate analysis. Kaplan-Meier survival curves based on median serum uric acid showed that patients with high values had a significantly worse survival rate than those with low values (log-lank test: p = 0.0014 in male patients, p = 0.0034 in female patients).
CONCLUSIONS—Serum uric acid increases in proportion to haemodynamic severity in adult patients with Eisenmenger syndrome and is independently associated with long term mortality.


     

Fatigue in primary Sj?gren's syndrome: Is there a link with the fibromyalgia syndrome?

OBJECTIVE—To determine whether fibromyalgia (FM) is more common in patients with primary Sjögren''s syndrome (pSS) who complain of fatigue. The association and prevalence of fatigue and FM was recorded in a group of patients with pSS and a control group of lupus patients, a subset of whom had secondary Sjögren''s syndrome (sSS).
METHODS—74 patients with pSS and 216 patients with lupus were assessed with a questionnaire to identify the presence of fatigue and generalised pain. From the lupus group, in a subset of 117 lupus patients (from the Bloomsbury unit) those with sSS were identified. All patients were studied for the presence of FM.
RESULTS—50 of 74 patients with pSS (68%) reported fatigue—a prevalence significantly higher than in the lupus group (108/216 (50%); p<0.0087). Fatigue was present in 7/13 (54%) patients with SLE/sSS. FM was present in 9/74 patients with pSS (12%), compared with 11/216 lupus patients (5%), and in none of the patients with SLE/sSS. None of these values corresponds with previously reported figures of the incidence of FM in pSS.
CONCLUSION—The results show that fatigue in patients with pSS and sSS is not due to the coexistence of FM in most cases. A lower incidence in the United Kingdom of FM in patients with pSS was found than has been previously reported.

     

SAPHO syndrome.

SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome put together osteoarticular lesions described separately under numerous denominations, such as multifocal osteomyelitis, pustulotic arthroosteitis, acne rheumatism. The association of sterile inflammatory bone lesions and neutrophilic skin eruptions is the cornerstone of this new syndrome, which also has links with spondyloarthropathies and plain psoriasis.     

"Value" of improved treadmill exercise capacity: lessons from a study of rate responsive pacing

Objective—To assess outcomes of pacemaker upgrade from single chamber ventricular to dual chamber.
Design—Retrospective analysis of patients undergoing the procedure.
Setting—Specialist cardiothoracic unit.
Patients—44 patients (15 female, 29 male), mean (SD) age at upgrade 68.2 (12.9) years.
Interventions—Upgrade of single chamber ventricular to dual chamber pacemaker.
Main outcome measures—Procedure duration and complications.
Results—Principal indications for upgrade were pacemaker syndrome (17), "opportunistic"—that is, at elective generator replacement (8), heart failure (7), non-specific breathlessness/fatigue (7), and neurally mediated syncope (3). Mean (SD) upgrade procedure duration (82.9 (32.6) minutes) significantly exceeded mean VVI implantation duration (42.9 (13.3) minutes) and mean DDD implantation duration (56.6 (22.7) minutes) (both p < 0.01). Complications included pneumothorax (1), ventricular arrhythmia requiring cardioversion (2), protracted procedure (10), atrial lead repositioning within six weeks (8), haematoma evacuation (1), superficial infection (1), and admission to hospital with chest pain (1); 20 patients (45%) suffered one or more complications including four of the eight who underwent opportunistic upgrade.
Conclusions—Pacemaker upgrade takes longer and has a higher complication rate than either single or dual chamber pacemaker implantation. This suggests that the procedure should be performed by an experienced operator, and should be undertaken only if a firm indication exists. Patients with atrial activity should not be offered single chamber ventricular systems in the belief that the unit can be upgraded later if necessary at minimal risk.

     

Severe inflammation associated with synovitis,acne, pustulosis,hyperostosis, osteitis (SAPHO) syndrome was markedly ameliorated by single use of minocycline

Abstract

We describe the case of a 63-year-old female who presented with severe inflammatory spondylitis, refractory to various antibiotics. Mycobacterial and fungal osteomyelitis were unlikely. Although asymptomatic, she also had osteomyelitis in the sternocostoclavicular region, and was suspected of having synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome, against which minocycline showed marked efficacy. The presence of severe inflammatory SAPHO, albeit rare, together with the marked efficacy of tetracycline, should be noted.     

Invasion and persistence of Salmonella in human fibroblasts positive or negative for endogenous HLA B27

OBJECTIVE—Analysis of the interaction of enteropathogenic bacteria with HLA B27 transfected murine fibroblasts showed a specific influence of HLA B27 on microbial invasiveness. This possible novel mechanism for the action of HLA B27 should be verified by using endogenous HLA B27 positive and negative human fibroblasts as a model for the direct interaction of arthritogenic bacteria and host cells.
METHODS—Fibroblasts were obtained from healthy donors positive or negative for HLA B27; cultivated as monolayers; and infected with Salmonella enterica serovar enteritidis.
RESULTS—Invasion and survival of bacteria in human cells was not influenced by the presence of HLA B27. Enhancement of HLA class I molecule expression by treatment of cultures with interferon gamma decreased invasion and survival of bacteria in both HLA B27 positive and negative cells. After disappearance of live bacteria lipopolysaccharide antigens persisted within cells.
CONCLUSION—Endogenous HLA B27 does not modulate the direct interaction of Salmonella with human cells. Non-professional phagocytes are able to limit bacterial survival in cells, and interferon gamma accelerates killing of bacteria, but arthritogenic antigens persist after disappearance of live bacteria.

     

Development of additional autoimmune diseases in a population of patients with systemic lupus erythematosus

OBJECTIVE—In view of the recognised clustering of autoimmune diseases (AID), the chronology of development of other autoimmune diseases in systemic lupus erythematosus (SLE) patients was considered.
METHODS—A retrospective review of a well documented population of 215 patients with SLE was undertaken. The duration of follow up ranged from 2 to 18 years.
RESULTS—Of these 215 patients, 65 (30%) had at least one other AID—including 51 (24%) having one other AID, 12 (6%) having two and two (1%) having three other AID in addition to their SLE. Twelve different autoimmune diseases were identified. The majority of patients developed a further AID after SLE had been diagnosed (62%) reflecting the relatively early age of onset of SLE. There was no significant difference in the age of onset of rheumatoid arthritis, Sjögren''s syndrome and hypothyroidism that had developed before SLE compared with those who developed these diseases after SLE. However, those who developed autoimmune thrombocytopenia (AITP) before SLE were significantly younger than those who developed AITP after SLE (16.7 v 38.7 years respectively, p<0.05).
CONCLUSIONS—Physicians caring for SLE patients should remain alert to the possible development of a second AID during follow up. Further well matched case-control studies are required to define the exact relation between SLE and other AID.

     

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.     

Incidence of rectosigmoid adenomatous polyps in subjects without prior colorectal adenoma or cancer: a prospective cohort study

BACKGROUND—Subjects without known colorectal adenomas or cancer constitute a large majority of the population where 85% of all cases of colorectal cancer are thought to occur. Consequently these people should be considered for screening to decrease mortality from colorectal cancer in the general population.
AIMS—To estimate the incidence rate of rectosigmoid adenomas in these subjects.
METHODS—Subjects without adenomas or cancer at a previous examination which had visualised the rectosigmoid underwent a fibre endoscopy every three years. Endoscopic data and population characteristics were collected prospectively.
RESULTS—A total of 450 subjects fulfilled the selection criteria; 287 (64%) underwent at least two examinations, and 163had three or more. At the second examination, with a mean delay of 39 months, the incidence rate of rectosigmoïd adenomas was 1.50% per patient year. The rate was 1.75% per patient year (95% CI 0.80-3.33) at the third endoscopy with an additional mean delay of 38 months. The cumulative incidence rate at six years was 7.3% (95% CI 4.3-10.3), representing a mean of 1.2% per patient year. This rate increased with age and was higher for men than for women after age adjustment (p< 0.03).
CONCLUSIONS—The incidence rates are very low compared with those of patients with prior adenomas. These results should be considered in establishing rectosigmoid adenoma screening strategies.

     

Ilium osteitis as the main manifestation of the SAPHO syndrome: response to infliximab therapy and review of the literature

OBJECTIVE: To analyze the clinical efficacy of anti-tumor necrosis factor (TNF)-alpha therapy in the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. We describe 2 new cases with ilium osteitis as the main SAPHO syndrome feature and review reported cases treated with anti-TNF-alpha. METHODS: A literature search of SAPHO syndrome cases treated with TNF-alpha blocking therapy with special emphasis on osteoarticular and skin responses was performed. RESULTS: Eighteen cases were identified: 17 SAPHO syndrome and 1 chronic recurrent multifocal osteomyelitis, a juvenile variant of SAPHO syndrome. Sixteen were reported cases and 2 were nonreported cases seen in our arthritis unit. Sixteen patients received infliximab and 2 received etanercept, with an early, sustained clinical improvement in most cases. CONCLUSIONS: Anti-TNF-alpha therapies are effective treatment for patients with refractory SAPHO syndrome, not only for cutaneous lesions but also for persistent bone lesions such as osteitis.