BackgroundTo update community-based prevalence values for Polymyalgia Rheumatic (PMR) and Giant Cell Arteritis (GCA) using case record review supplemented by population survey and subsequent clinical review.MethodsClinical data were obtained from case records of a large primary care practice in Norfolk, UK and reviewed for diagnoses of GCA and PMR. In addition postal survey was carried out to capture potentially undiagnosed cases within the practice population. Those screening positive for potential diagnoses of GCA and PMR were invited for clinical review. A cumulative prevalence estimate was subsequently calculated on those diagnosed within the GP practice and subsequently on those fulfilling the various published classification criteria sets. The date of the database lock and mail merge was March 2013.ResultsThrough detailed systematic review of 5,159 GP case records, 21 patients had a recorded diagnosis of GCA and 117 had PMR.No new cases were identified among 2,227 completed questionnaires returned from the population survey of a sample of 4,728. The resulting cumulative prevalence estimate in those aged ≥ 55 years meeting the ACR classification criteria set for GCA was 0.25 % (95 % CI 0.11 to 0.39 %) and for five published criteria sets for PMR ranged from 0.91 to 1.53 % (95 % CI ranges 0.65 %, 1.87 %). The prevalence of both conditions was higher in women than in men and in older age groups.ConclusionThis study provides the first UK prevalence estimate of GCA and PMR in over 30 years and is the first to apply classification criteria sets.

Objective The aim of this study was to survey participants with PMR to evaluate the face validity, acceptability and domain match of proposed candidate outcome measures. Methods A structured, online, anonymous survey was disseminated by patient support groups via their networks and online forums. The candidate outcome measures comprised: 1. visual analogue scale (VAS), numerical rating score (NRS) to assess pain; 2. VAS, NRS and duration to assess stiffness; 3. the modified Health Assessment Questionnaire (mHAQ) and Health Assessment Questionnaire Disability Index (HAQ-DI) to assess physical function; 4. C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) to assess inflammation. Free text answers were analysed using descriptive thematic analysis to explore respondents' views of the candidate instruments. Results Seventy-eight people with PMR from six countries (UK, France, USA, Canada, Australia and New Zealand) participated in the survey. Most respondents agreed candidate instruments were acceptable or “good to go”. Free text analysis identified five themes which participants felt were inadequately covered by the proposed instruments. These related to: (i) the variability, context and location of pain, (ii) the variability of stiffness, (iii) fatigue, (iv) disability, and (v) the correlation of inflammatory marker levels and severity of symptoms, sometimes reflecting disease activity and other times not. Conclusion Participants reported additional aspects of their experience which are not covered by the proposed instruments particularly for the experience of stiffness and impact of fatigue. New patient-reported outcome measures are required to increase the relevance of results from clinical trials to patients with PMR.

Objective ANCA-associated vasculitis (AAV) is a rare multisystem disease. Modern therapeutic protocols have turned AAV from an acute, frequently fatal disease into a chronic disease requiring long-term immunosuppression. Patients must often manage substantial burdens related to chronic illness and treatment-related side effects, requiring help from informal carers. This study aimed to explore the experience of patients and of informal carers of patients with AAV about the impact of managing a rare rheumatic condition. Methods A qualitative approach using semi-structured interviews was used. Interviews were conducted with a purposeful sample of 18 pairs of patients with AAV and their informal carers. The interviews were used to explore the participants’ experience and effects of caring. The interviews were recorded and transcribed as verbatim text and analysed using the framework technique. Results Eighteen patients (seven female; disease: 10 granulomatosis with polyangiitis; four microscopic polyangiitis; four eosinophilic granulomatosis with polyangiitis; age range 34–78 years; disease duration 1–20 years). Caregiver and patient perspectives were shared. The emerging themes were the physical and psychological impacts of the disease, the need for constant vigilance and fear of the future. Conclusion Both patients and carers faced a range of challenges in managing a rare condition, including the seriousness of the illness, dealing with the emotional toll and knowing what to expect. This study offers insight into the experiences of patients and informal carers, and health-care professionals should address individuals’ fears and expectations for recovery.

Brief reports 4 7 7ruled out because of the absence of invasive glands and cytological atypia. The differential diagnosis can be more difficult with inflammatory fibrosarcoma, which, on some occasions, has been called inflammatory pseudo-tumour3. The present case lacked the cellularity and the pleomorphic nuclei with prominent nucleoli commonly seen in inflammatory fibrosarcoma. Moreover, the pancrease was not affected in any of the cases described by Meis and Enzinger in their review of this entity3. Malignant lymphoma was not considered to be a diagnostic problem because of the polymorphism of the cell infiltrate, the absence of cytologic atypia and the polyclonality of the mononuclear cells as detected by immunohistochemistry .The only other two reported cases of inflammatory pseudotumour of the pancreas that we were able to find in the literature were described in ~h i l d r e n~,~. Neither had recurrence of the lesions following surgery4e5. Our patient has been asymptomatic for 6 months after surgery. References1 . Pettinato G. Manivel jC. De Rosa N. Dehner Lp. Inflammatory myofibroblastic tumor (plasma cell granuloma). Clinicopathologic study of 2 0 cases with immunohistochemical and ultrastructural observations. Am. /. Clin. Puthol. 1990: 94: 538-546. 2. Hurt MA. Santa Cruz 111. Cutaneous inflammatory pseudotumor. Lesions resembling 'inflammatory pseudotumors' or 'plasma cell granulomas' of extracutaneous sites. Am. /. Surg. Puthol. 1990: 14: 764-773. 3. Meis JM. Enzinger FM. Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor.

No abstract