ANTERIOR MEDIASTINAL FIBROSIS WITH SUPERIOR VENA CAVAL OBSTRUCTION COMPLICATING THE SYNOVITIS--ACNE--PUSTULOSIS--HYPEROSTOSIS--OSTEOMYELITIS SYNDROME

A syndrome of predominantly palmo-plantar pustular psoriasisassociated with anterior chest wall (sternoclavicular, manubriosternal)inflammation has previously been described. In this case, afurther patient is reported where chest wall involvement iscomplicated by anterior mediastinal fibrosis and superior venacaval obstruction. KEY WORDS: Mediastinal fibrosis, superior vena caval obstruction, Synovitis-acne-pustulosis-hyperostosis-osteomyelitis syndrome     

Unexplained Syncope—A Diagnostic Pacemaker?

We describe our initial experience with a pacemaker which performs the dual function of registering episodes of cardiac arrest and pacing the heart when necessary. The appartus has a prolonged escape interval (2.30–2.65 seconds) and is capable of counting up to 128 "events." Twenty-five patients complaining of infrequent episodes of loss of consciousness have been studied. Blackouts ceased and episodes of ventricular standstill were recorded in 14 cases; the unit helped to establish a non-cardiac etiology for the attacks in two cases. It is suggested that a bradycardia-recording facility incorporated into a programmable pacemaker would provide a much needed tool in the diagnosis and management of patients with infrequent episodes of loss of consciousness in whom a cardiac mechanism is suspected but unproven.     

Immunolocalization of adhesion molecules in psoriatic arthritis, psoriatic and normal skin

Adhesion molecule expression in synovial membrane obtained from patients with psoriatic arthritis (PA) has previously been compared with rheumatoid arthritis (RA). Although expression of intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) was similar in both psoriatic and rheumatoid synovium, in contrast, little or no endothelial leucocyte adhesion molecule-1 (ELAM-1) was observed in psoriatic synovium. In the present study, the expression of ICAM-1. ELAM-1 and VCAM-1 was examined in the involved and uninvolved skin from patients with PA (n= 15), patients with psoriasis (Ps) but no arthritis (n= 5) and in normal skin (n= 4). ICAM-1 was intensely expressed on endothelium and keratinocytes of involved skin from patients with Ps with or without arthritis. There was constitutive expression of ICAM-1 on endothelium only in uninvolved and normal skin. In contrast, ELAM-1 expression was restricted to endothelial cells; it was widespread and intense in involved skin, but was minimal in uninvolved and normal skin. VCAM-1 was expressed on endothelium, and also on some dendritic cells in involved psoriatic skin. There was minimal VCAM-1 staining on endothelial cells in uninvolved and normal skin. In conclusion, in involved psoriatic skin from patients with and without arthritis ICAM-1, ELAM-1 and VCAM-1 expression is up-regulated on vascular endothelium, and ICAM-1 is expressed on keratinocytes. However, ELAM-1 and VCAM-1 expression seen in dermal vessels is not found in psoriatic synovial vessels. These differences suggest a mechanism for controlling cellular traffic in Ps and in PA.     

Exercise Dependence

Exercise can become a compulsive behaviour and harmful to an individual. This review proposes diagnostic criteria for ‘exercise dependence’ to facilitate recognition in Sports clinics and further research. The importance of diagnosing exercise dependence lies in the prevention of morbidity and rarely mortality if exercise is continued in the presence of illness or injury. There is insufficient evidence to postulate opioid peptides as a physiological basis of dependence. A distinction is made between a primary form of exercise dependence and that which is secondary to an eating disorder.     

Tussometry and lung function

The tussometric parameter peak value time (PVT) has been proposed as an objective measure of laryngeal function. Whether the lower respiratory tract function influences its value is, however, unknown. This study investigates the relationship between lung function parameters forced expiratory volume in 1 second, vital capacity, peak expiratory flow rate, forced expiratory flow at 50% of vital capacity and total lung capacity (FEV₁ VC, PEFR, FEF₅₀, TLC) and PVT in 79 subjects. PVT proved to be independent of lung function. It is therefore specifically a measure of laryngeal function, and lung function need not be considered when interpreting PVT values.     

PRIMARY FIBROMYALGIA AND THE IRRITABLE BOWEL SYNDROME: DIFFERENT EXPRESSIONS OF A COMMON PATHOGENETIC PROCESS

Primary fibromyalgia (PFM) and the irritable bowel syndrome(IBS) are both common conditions which account for 30% or moreof referrals to rheumatology and gastroenterology clinics. Anassociation between symptoms in PFM and IBS has been suggestedbut the frequency with which they coexist has not been assessed.The aim of this study was to examine the prevalence of eachcondition in groups of patients with PFM and IBS compared tonormal and disease control populations. We studied four patient groups, 20 patients in each group, withPFM, IBS, inflammatory arthritis, inflammatory bowel diseaseand also 20 normal controls. Using strict diagnostic criteria,each group was assessed by two investigators for symptoms andsigns of PFM and IBS. Sigmoidoscopy was performed when indicated.Results indicate that 70% (14/20) of the PFM patients had IBSand 65%(13/20) of the IBS patients had PFM. This compared withthe control groups where 12%(7/60) and 10%(6/60) had PFM andIBS respectively. In conclusion, these results indicate that PFM and IBS frequentlycoexist. A common pathogenetic mechanism for both conditionsis therefore suggested. KEY WORDS: Frequency, Colonic dysfunction, Fibrositis     

Medical thoracoscopy in the diagnosis of unexplained pleural effusion

Abstract Approximately 20% of pleural effusions remain without an established aetiology after evaluation. Thoracoscopy has a very high sensitivity for the diagnosis of both benign and malignant diseases and greatly increases the diagnostic yield for pleural effusion. We sought to evaluate the diagnostic yield and safety of medical thoracoscopy at this institution. The records of all patients undergoing medical thoracoscopy for the evaluation of undiagnosed pleural effusion between 1990 and 1996 were reviewed. The procedure was performed under local anaesthesia with sedation using a Stortz rigid thoracoscope. Fifty-eight patients had thoracoscopy, most having had two (range: 1–6) non-diagnostic pleural aspirations and biopsies of the pleura. Nineteen patients were found to have mesothelioma and nine metastatic malignancy. Three patients were considered likely to have tuberculous pleural disease, six had asbestos related benign pleural fibrosis and three post-cardiotomy syndrome. There was one chylous effusion of uncertain aetiology, one post-traumatic and two other benign effusions, both of which resolved without clear aetiology. On seven occasions the pleural space could not be adequately accessed, but none of these patients had prior computerized tomography (CT) or ultrasound of the pleural space. There were five false negative diagnoses of malignancy, but no false positives. The diagnostic sensitivity for pleural malignancy was 85% and specificity 100%. There were no major complications, but four patients had late tumour seeding at the thoracoscopy site. Medical thoracoscopy is a safe procedure with a high diagnostic yield. Pre-operative evaluation of the pleural collection using ultrasound or CT increases the likelihood of successful access to the pleural space and may increase diagnostic yield.     

SULPHASALAZINE-INDUCED LUPUS IN PSORIATIC ARTHRITIS

Lupus syndromes have been described in rheumatoid arthritisand inflammatory bowel diseases treated with sulphasalazine.We describe two cases of lupus with clinical and serologicaldisease, developing in seronegative, immunologically nude psoriaticarthritis following sulphasalazine treatment. On cessation ofsulphasalazine the clinical and serologic features of lupusdisappeared. This suggests that the lupus syndrome was inducedby sulphasalazine rather than unmasked by it. KEY WORDS: Sulphasalazine, Lupus, Psoriatic arthritis     

Breathing During Sleep in Patients with Myotonic Dystrophy and Non-myotonic Respiratory Muscle Weakness

Sleep apnoea and hypopnoea have been reported in myotonic dystrophy,but it is unclear whether this is simply attributable to therespiratory muscle weakness which is common in this condition.We therefore investigated whether breathing and oxygenationduring sleep were more abnormal in patients with myotonic dystrophythan in patients with non-myotonic muscle weakness. Seven subjectswere studied in each of three groups: normal controls, myotonicdystrophy and non-myotonic weakness. Patients in the lattergroup were chosen to represent a similar range of severity ofrespiratory muscle weakness to those with myotonic dystrophy.Detailed polysoranography was performed; the severity of breathingdisorders during sleep was quantified in terms of the frequenciesof apnoea and hypopnoea and the degree of arterial desaturation.The myotonic patients showed more frequent apnoea and hypopnoeaand more severe desaturation than the other two groups; theresults in the non-myotonic patients were generally intermediate.The results suggest that abnormal breathing during sleep iscommon in myotonic dystrophy and is not due solely to the directeffects of respiratory muscle weakness. Somnolence, which isa well recognized symptom of myotonic dystrophy, was not clearlyattributable to the sleep apnoea/hypopnoea syndrome nor to abnormalsleep architecture in the myotonic patients.