Giant cell aortitis leading to Stanford type B and type A aortic dissection.

This report provides a rare histological example and the appropriate management of spontaneous aortic dissection secondary to giant cell arteritis.

Diagnosis and assessment of systemic vasculitis.

The diagnosis of systemic vasculitis requires clinical evidence with appropriate symptoms and physical signs, supported by histological or radiological confirmation. Earlier recognition of these diseases has been facilitated by a greater awareness of their incidence, and also by the more widespread introduction of the anti-neutrophil cytoplasmic antibody (ANCA) test. Early diagnosis provides a greater potential for effective intervention in the course of disease and this may limit subsequent damage. However, an early diagnosis poses the more difficult challenge in the classification of the vasculitides, since traditional classification systems have depended on the presence of well-established manifestations of the disease. The accurate assessment of disease activity and damage in vasculitis has become necessary as a result of significant improvements in survival with the use of chemotherapy. The disease course however is frequently characterised by relapse as well as the scars of irreversible organ damage from disease and drug toxicity. Clinical methods of assessment are simple to apply, reliable and often more effective than any current laboratory test in evaluating the effects of therapy and determining changes in therapy. The increasing use of surrogate clinical measures of disease should provide a greater opportunity to establish the effectiveness of existing and novel therapies in the management of these complex diseases.

The diagnostic value of anti-neutrophil cytoplasmic antibody testing in a routine clinical setting.

Anti-neutrophil cytoplasmic antibody (ANCA) tests are a routine clinical assay in most UK hospitals. We examined the role of routine ANCA testing in achieving a diagnosis of systemic vasculitis in a routine clinical setting. From April 1996 to March 2000, 2734 samples from five hospital departments were tested for ANCA by indirect immunofluorescence (IIF) at a single laboratory. After April 1999, enzyme-linked immunosorbent assays (ELISAs) were performed on all IIF-positive samples. Clinical diagnosis was determined for all patients with a positive IIF ANCA, and a sample of the ANCA-negative patients. Some 2-18% of patients with suspected ANCA-associated systemic vasculitis (AASV) had positive IIF ANCA. The AASV diagnosis was confirmed in 0-56% of these cases. Analysis by department suggested that 88-100% of patients with a positive IIF ANCA did not have AASV, except in the Rheumatology department. The positive predictive value (PPV) of IIF ANCA for AASV was 59% and the negative predictive value (NPV) was 84%. Of the patients with proven AASV, 41% did not have ANCA on IIF. Combined ANCA testing by IIF/ELISA had a higher sensitivity and PPV but lower specificity than IIF alone for AASV. For the combined IIF/ELISA test, only the Rheumatology department had a sensitivity or PPV >0% for AASV. The PPV of ANCA by IIF/ELISA for AASV was 79% and the NPV was 63%. The ANCA test is being widely applied with very poor return. Guidelines for more effective usage are proposed.

The assessment and management of leg ulcers in rheumatoid arthritis.

Patients with rheumatoid arthritis appear to be at increased risk of developing chronic leg ulcers. This review identifies the factors that predispose these patients to leg ulceration and highlights how this problem can be managed and the risk of recurrence reduced.

Successful treatment of severe muscle necrosis with intravenous immunoglobulin.

Intravenous immunoglobulin (IVIG) is an effective treatment for a range of conditions believed to have an autoimmune basis, including inflammatory muscle disease. We describe a patient with muscle disease characterized by severe necrosis without clinical or biopsy evidence of inflammation or vasculitis, who responded to treatment with IVIG.

The short-term health outcome of out-patient rheumatology consultations in relation to rationing: a pilot study.

The objectives were to test whether the short-term health outcome of rheumatology out-patients differs according to clinical priority. The setting was an NHS regional rheumatology out-patient department serving a catchment population of over 1 million. The subjects were 249 consecutive rheumatology out-patients categorized on the basis of the referral letter as 'urgent' (n = 50), 'soon' (n = 100) or 'routine' (n = 99). Primary outcome measures were the proportion of patients reporting improvement in health categorized by clinical priority (urgent, soon or routine) or main diagnostic group (inflammatory or non-inflammatory disease). Secondary outcome was change in health status measured using the EuroQol generic health instrument (EQ-5D). Small but insignificant differences in the proportion of patients reporting health improvement were found between the urgent (28%), soon (23%) and routine (17%) categories (Kruskal-Wallis, P = 0.186). Thirty per cent of patients with inflammatory joint disease reported improvement compared with 17% of those with non-inflammatory conditions (Mann-Whitney U, P = 0.019). In patients reporting improvement, the median (interquartile range) improvement in EQ-5D health utility score was +0.2 (0.58) (P = 0.0001) and that of visual analogue health score was +5 (16) (P = 0.001). Clinical priority setting, by giving priority to some patients over others, results in rationing by delay. These data do not support the hypothesis that fewer patients given a low clinical priority gain health benefit compared with those given a high priority. However, those with inflammatory joint disease do appear to have better short-term health outcomes.

The relevance of large-vessel vascular disease and restricted ankle movement to the aetiology of leg ulceration in rheumatoid arthritis.

Leg ulceration in rheumatoid arthritis (RA) without systemic vasculitis is a difficult clinical problem and a common cause of morbidity. We have assessed venous function, arterial pressures and range of ankle movement in 23 RA patients with a leg ulcer and compared the results with those in the non-ulcerated contralateral limb and in 25 RA patients matched for age and duration of arthritis. We found evidence of venous insufficiency in RA ulcer patients compared to disease controls. Ankle movement was more restricted in the ulcerated limb compared to the non-ulcerated contralateral leg. There was no difference in large-vessel arterial function between groups. These findings have implications for therapy and rates of healing.

Rheumatoid constrictive pericarditis.

A 73-yr-old woman with a 4 yr history of rheumatoid arthritis presented with the clinical features of congestive cardiac failure. She had a good early response to standard therapy although she subsequently developed recurrent biventricular failure. The preservation of good ventricular function on echocardiography in the face of clinical evidence of myocardial insufficiency raised the possibility of constrictive pericarditis, which was confirmed on cardiac catheterization. Constrictive pericarditis should be considered in patients with rheumatoid arthritis who develop unexplained cardiac failure. Early diagnosis requires a high index of suspicion and cardiac catheterization may be necessary to confirm the diagnosis. Medical treatment is largely ineffective and pericardiectomy should be considered.

Cervical spine surgery in patients with rheumatoid arthritis: an appraisal.

OBJECTIVES: To review the outcome of surgery undertaken to stabilise the neck in patients with rheumatoid arthritis performed over a five year period, to compare the results with those of previous reports, and to identify factors that may predict surgical outcome. METHODS: Outcome was assessed at time of discharge from hospital after surgery by review of patients' notes, and at follow up by patient interview, clinical examination, anonymous questionnaire, and cervical spine radiograph. The Ranawat classification of neurological impairment and Steinbrocker functional classification were used. RESULTS: Thirty nine patients underwent 44 procedures; 28 patients were available for review after a mean period of 29.8 months (range 12-65 months). Fourteen patients had preoperative neurological impairment and were available for follow up; 13 returned the questionnaire. Four (29%) had improved Ranawat class, nine were unchanged, and one had deteriorated. Nine (69%) reported a subjective improvement in neurological symptoms by questionnaire, even though the Ranawat class was unchanged in five. Twenty five of the patients reviewed had pain before operation; 21 returned the questionnaire. Pain relief was reported by direct questioning and questionnaire in 76% and 67% of patients, respectively. Overall, 67% felt that surgery had been successful. Surgery was more successful in producing symptomatic relief in patients with neck or radicular pain than in those with neurological deficit, but did prevent progression of neurological symptoms. CONCLUSIONS: Our results are similar to those from other centres. Overall patient satisfaction with surgery was good. Surgery was more likely to produce symptomatic relief in patients with neck or radicular pain before operation than in those with neurological deficit. The greater subjective improvement in neurological symptoms as judged by questionnaire probably reflects the relative insensitivity of the Ranawat classification in detecting change in neurological status; previous reports of poor outcome for patients with neurological symptoms who undergo surgery may in part be a reflection of the insensitivity of this method of assessment. No clear factors emerged which allowed prediction of those patients at greatest risk of operative mortality. In particular, an increased risk of neurological compromise appeared to confer no additional risk of immediate perioperative death. Our data support the suggestion that early surgery to correct symptomatic atlantoaxial subluxation may prevent progression of instability.

Leg ulceration in rheumatoid arthritis.

Chronic leg ulcers occur in 1% of the adult population with considerable associated morbidity, and community costs estimated at up to 600 million pounds p.a. in the UK. Leg ulcers appear to be relatively common in patients with RA and are widely believed to be resistant to treatment. Such ulcers are often attributed to vasculitis, but little is known of the occurrence of other potential aetiological factors. This article reviews the epidemiology, natural history and aetiology of chronic leg ulcers in RA as well as the therapeutic options available.

Lingual infarction in cranial arteritis.

An unusual case is described of infarction of the tongue as a presentation of cranial arteritis. The lingual necrosis may have been precipitated in part by the use of ergotamine to relieve the headache of the temporal arteritis, mistaken initially for migraine. The patient recovered with high-dose steroid therapy.

Riedel's thyroiditis complicated by hypoparathyroidism and hypothyroidism.

Riedel's thyroiditis is a rare condition in which the thyroid gland is replaced by fibrous tissue. The case reported is one of Riedel's thyroiditis complicated by hypoparathyroidism and hypothyroidism. There would appear to have been only five earlier reports in the literature of hypoparathyroidism complicating Riedel's thyroiditis.