Disease activity in longstanding ankylosing spondylitis: a correlation of clinical and magnetic resonance imaging findings.

We evaluated magnetic resonance imaging (MRI) changes in ankylosing spondylitis (AS) patients with longstanding disease and investigated whether there is any relationship between MRI findings and validated methods of disease assessment. A total of 34 AS patients with disease duration greater than 10 years were included in this observational cross-sectional study (26 men, 8 women). The main outcome measures were Bath Ankylosing Spondylitis Functional Index (BASFI), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Global assessment (BASG), Bath Ankylosing Spondylitis Metrology Index (BASMI), MRI of the thoracic and lumbar spine (AS spi MRI A) and measurement of serum erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), plasma viscosity (PV) and immunoglobulin A (Ig A). The median scores for the acute lesions based on AS spi MRI A scoring system was 2.5 (0-4.12). The respective mean ESR and CRP were 36 (SD, 24.00) mm/h and 14.19 (SD, 24.00) mg/l with the median PV of 1.8 (1.75-1.87). The median BASG, BASFI and BASDAI were 4.55 (2.37-5.55), 4.40(2.31-5.47) and 4.32 (3.07-6.48), respectively. No significant correlations were found between the acute MRI scores and each of the clinical instruments and laboratory markers of inflammation. In this study, majority of AS patients with longstanding disease had very low AS spi MRI A scores or no evidence of spinal inflammatory lesions. Our study would suggest that MRI should be used along with other measures of disease activity in the assessment of symptomatic AS patients with longstanding disease.

Prevention of vascular damage in scleroderma and autoimmune Raynaud's phenomenon: a multicenter, randomized, double-blind, placebo-controlled trial of the angiotensin-converting enzyme inhibitor quinapril.

OBJECTIVE: To evaluate the efficacy and tolerability of prolonged administration of quinapril, a long-acting angiotensin-converting enzyme inhibitor, in the management of the peripheral vascular manifestations of limited cutaneous systemic sclerosis (lcSSc) and in the prevention of the progression of visceral organ involvement in the disease. METHODS: This was a multicenter, randomized, double-blind, placebo-controlled study evaluating quinapril 80 mg/day, or the maximum tolerated dosage, in 210 patients with lcSSc or with Raynaud's phenomenon (RP) and the presence of SSc-specific antinuclear antibodies. Treatment was for 2-3 years. The primary outcome measure was the number of new ischemic ulcers appearing on the hands; secondary measures were the frequency and severity of RP attacks, skin score, treatments for ischemia, health status (measured by the Short Form 36 instrument), measures of kidney and lung function, and echocardiographic estimates of pulmonary artery pressure. An intent-to-treat analysis was used. RESULTS: Quinapril did not affect the occurrence of digital ulcers or the frequency or severity of RP episodes. It did not alter the treatments that were prescribed for either infected ulcers or severe RP symptoms. There was no apparent effect on the estimated tricuspid gradient. Health status was not affected by quinapril, and one-half of the patients who believed they had benefited from the trial treatment were in the placebo arm. Quinapril was not tolerated by one-fifth of the patients, with dry cough being the most frequent side effect. CONCLUSION: Administration of quinapril for up to 3 years had no demonstrable effects on the occurrence of upper limb digital ulcers or on other vascular manifestations of lcSSc in this patient population.

Tear lipid layer thickness and ocular comfort with a novel device in dry eye patients with and without Sjögren's syndrome.

BACKGROUND: To measure changes in tear-film lipid-layer thickness (LLT) and symptoms in patients with dry eye symptoms with and without Sjögren's syndrome after using a novel device. The device is designed to promote release of meibomian sebum into the tear film by delivering latent heat to the eyelids. STUDY DESIGN: Two prospective, controlled, randomised, observer-masked, single-intervention studies. METHODS: Two independent studies were conducted in a major university hospital in the South West of England. The first study involved 24 patients with dry eye symptoms without Sjögren's [the PDE study] and the second study involved 31 patients with dry eye symptoms and Sjögren's syndrome (the SS study). The PDE study was randomised into two groups. Group I (12 patients) underwent 10 min of treatment with the activated device and Group II (12 patients) had no treatment. The SS study was similarly randomised into Group I (17 patients) and Group II (14 patients). The LLT and subjective alterations in ocular comfort of each subject were assessed prior and immediately after 5 and 30 min subsequent to the 10-min period. In the SS study, a further assessment was carried out at 60 min. RESULTS: In the PDE study, treated patients exhibited a bilateral increase of LLT at 5 min (right eyes, 1.2 levels, p<0.0005; left eyes, 1.0 levels, p<0.0005, Mann-Whitney) and at 30 min (right eyes, 0.7 levels, p<0.005; left eyes, 0.6 levels, p<0.005). Mean symptom scores improved in the treated group compared with the control group at 5 min (treatment group, +2.0; control group, +0.2; p<0.05) and 30 min (treatment group, +2.8; control group, +0.4; p<0.015). In the SS study, treated patients exhibited a bilateral increase of LLT, 5 min (right eyes, 0.5 levels, p<0.009; left eyes, 0.5 levels, p<0.005, Monte Carlo 2-tailed), 30 min (right eyes, 0.5 levels, p<0.007; left eyes 0.5 levels, p<0.002) and 60 min (right eyes, 0.3 levels, p<0.1; left eyes, 0.3 levels, p<0.05). There was no change in any of the control patients in any of the assessments. With regard to symptom scores, the mean change at 5 min measured +0.8 in the treatment group and remained relatively unchanged at +0.1 in the control group (p<0.1). At 30 min, this change measured +1.3 in the treatment group and +0.1 in the control group (p<0.03) and at 60 min, the change measured +1.5 in the treatment group and remained at +0.1 in the control group (p<0.02). CONCLUSION: Meibomian therapy with this novel device increases LLT and ocular comfort in patients with dry eye symptoms with and without Sjögren's syndrome.

Candida albicans peritonitis in a patient with Felty's syndrome.

A 53 year old man with Felty's syndrome presented with abdominal pain and fever. He underwent a laparotomy after starting broad spectrum antibiotics. An intestinal biopsy showed skip ulcers with fungal hyphae. Peritoneal exudates grew Candida albicans. He was started on intravenous fluconazole and then switched to liposomal amphotericin to which he showed a good clinical response. After one month at home he was readmitted with candidosis and died of a myocardial infarction.

Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline.

OBJECTIVES: To evaluate the effect of minocycline as treatment for cutaneous calcinosis in limited cutaneous systemic sclerosis (lcSSc). METHODS: Patients with lcSSc who had cutaneous calcinosis causing pain or ulceration, or both, were prescribed minocycline 50 or 100 mg daily regularly in an open label manner between November 1994 and April 2000. At routine clinical follow up the appearance of the calcinosis deposits was assessed clinically and radiographically, and the patients' assessment of the degree of discomfort, size, and frequency of ulceration was recorded. Demographic data, including disease duration, clinical features, and antinuclear antibody (ANA) titres, were also recorded. RESULTS: Nine patients have been treated to date. Eight of the nine patients were ANA positive, five of whom were positive for anticentromere antibodies. Eight patients have shown definite improvement and seven patients continue to receive treatment. The frequency of ulceration and inflammation associated with the calcinosis deposits decreased with treatment. The size of the calcinosis deposits also decreased but was less dramatic than expected. Improvement occurred at the earliest after one month of treatment with a mean (SD) of 4.8 (3.8) months. The mean (SD) length of treatment was 3.5 (1.9) years. An unexpected effect was the darkening of the calcinosis deposits to a blue/black colour. CONCLUSIONS: Minocycline may be effective in the control of calcinosis in systemic sclerosis. A low dose only is required and appears to be generally well tolerated. The mechanism of action may be mainly through inhibition of matrix metalloproteinases and anti-inflammatory effects. Calcium binding properties and antibacterial actions may also have a role.

Postpartum multifocal avascular necrosis: what are the possible etiologies?

Avascular necrosis of bone (osteonecrosis) that is atraumatic is most frequently associated with corticosteroid excess or alcoholism and usually involves the femoral head. We report a case of multifocal avascular necrosis in a 38-year-old woman with autoimmune Addison's disease taking corticosteroid replacement therapy. The onset of joint symptoms occurred 6 months after a pregnancy complicated by acute fatty liver and disseminated intravascular coagulation. Although both knees and ankles were involved, an unusual feature is that the hips were spared. As illustrated in this patient, avascular necrosis is frequently misdiagnosed in cases of joint pain of acute onset and may occur in the context of physiologic replacement doses of corticosteroids. Etiologic factors can precede the onset of symptoms and the diagnosis by several months.

Generalized morphoea in a patient with Felty's syndrome.

We describe a 54-year-old woman with long-standing rheumatoid arthritis complicated by Felty's syndrome and lung involvement who developed generalized morphoea with no features of systemic sclerosis. To our knowledge this is the first description of such a case.

Joint destruction after glucocorticoids are withdrawn in early rheumatoid arthritis. Arthritis and Rheumatism Council Low Dose Glucocorticoid Study Group.

OBJECTIVE: Prednisolone reduced the progression of joint destruction over 2 yr in early, active rheumatoid arthritis. The response to discontinuation of prednisolone under double-blind conditions is now reported. METHODS: A randomized, double-blind, placebo-controlled trial of prednisolone 7.5 mg daily in addition to routine medication over 2 yr in 128 patients with early rheumatoid arthritis, using radiological progression (changes in the Larsen score) and the development of erosions as primary outcome measures. Study medication was blindly discontinued and follow-up maintained for a further year. Other assessments included disability, joint inflammation, pain and the acute-phase response. RESULTS: Similar results were obtained when all available radiographs were included for each year of assessment (maximum 114) and when only patients with radiographs at all time points were included (75 patients). In these 75, the mean progression in the prednisolone group was 0.21 Larsen units in year 1, 0.04 units in year 2 and 1.01 units in year 3 (P = 0.587, 0.913 and 0.039 for change within each year, respectively). The equivalent placebo group means were 2.34, 1.00 and 1.63 Larsen units (P = 0.001, 0.111 and 0.012; difference between groups: 2.13, 0.96 and 0.67 units, P = 0.082, 0.02 and 0.622). The percentage of hands which had erosions at each time point was: prednisolone group: 27.8, 29.2, 34.7 and 39.2; placebo group: 28.2, 48.7, 59.0 and 66.5. There was little evidence for a flare in clinical symptoms after discontinuation of prednisolone. CONCLUSION: Joint destruction resumed after discontinuation of prednisolone. This corroborates the previously reported therapeutic effect and challenges current concepts of disease pathogenesis.

Do the radiological changes of classic ankylosing spondylitis differ from the changes found in the spondylitis associated with inflammatory bowel disease, psoriasis, and reactive arthritis?

OBJECTIVE: In 1971 McEwen and colleagues suggested that the radiological changes of classic ankylosing spondylitis (AS), and the changes of the spondylitis associated with inflammatory bowel disease differ in several respects from the radiological features of psoriatic and reactive spondylitis. The findings of this study have never been confirmed. The aim of this study was to replicate the McEwen study comparing films blinded to diagnostic group. METHODS: The study population comprised 91 patients with classic AS, 15 patients with regional enteritis, 16 patients with ulcerative colitis, five patients with sexually acquired reactive arthritis, two with post-dysenteric arthritis, and 34 with psoriatic arthritis. Blinded reading of spinal radiographs was undertaken, scoring for severity, symmetry, paravertebral ossification, size of syndesmophytes, ligamentous calcification, squaring, discitis, pseudofractures, zygoapophyseal joint involvement, and complete ankylosis. RESULTS: Comparison of the four groups--classic, enteropathic, psoriatic, and reactive AS--showed differences with respect to symmetry of sacroiliitis, symmetry of lumbar spinal involvement, and frequency and size of syndesmophytes. Zygoapophyseal joint involvement was more frequent in the lumbar spine in classic and enteropathic spondylitis but no between group differences were found with respect to symphisitis, squaring, apophyseal joint involvement and ligamentous calcification in the lumbar spine, and other areas. CONCLUSIONS: Some of the radiological differences described by McEwen et al, notably the asymmetry, the less severe changes, and the distinctive syndesmophytes in psoriasis, have been confirmed. A number of phypotheses are proposed to explain these differences including biomechanical, biochemical, and genetic factors.

Familial dermatomyositis.

A case of familial dermatomyositis (DM) is reported in a 27-yr-old mother and her daughter aged 2 yr and 8 months. Familial DM is a rare occurrence and has never been reported in a child of < 4 yr old, although isolated cases of DM have been found in infants as young as 1 yr. The mother responded readily to corticosteroids; however, the child proved resistant to steroids and was eventually successfully managed on a combination of methotrexate and prednisolone. Another daughter presented with the dermatological stigmata of DM, but with no evidence of muscle involvement.

Thrombosis associated with antiphospholipid antibody in juvenile chronic arthritis.

Anticardiolipin antibodies (aCL) have been reported in patients with juvenile chronic arthritis (JCA) without associated thrombotic events. We describe a patient with longstanding JCA who developed central retinal vein occlusion in the presence of antiphospholipid antibodies (APL).