Interstitial Lung Disease in Rheumatoid Arthritis: A Clinical Dilemma

Rheumatoid arthritis (RA)–associated interstitial lung disease (RA-ILD) is an increasingly common extra-articular cause of mortality and morbidity in RA. Here, we present the case of an 82-year-old female suffering from RA-ILD. She presented with a 20-year history of RA with new-onset progressively worsening dyspnoea, pyrexia, and dry cough, which were not responsive to initial antibiotics. Moreover, she had been on long-term methotrexate, which can cause pulmonary disease. Investigations revealed raised inflammatory markers, restrictive lung patterns, and radiological features of ILD. She was treated with a tapering course of oral prednisolone and home oxygen, which provided some symptomatic improvement. Diagnosis of RA-ILD can be challenging due to several contributing factors.  Optimal treatment is controversial, and corticosteroids have been widely used but with limited effects. There is evidence directed at potential therapeutic benefit from a number of newer agents, which are discussed. 

Diagnosis & Management of Vitamin B12 Deficiency in Primary Care – Are we following the guidelines?

Background: Vitamin B12 deficiency is common in primary care but its treatment practices vary across centres. One important cause of B12 deficiency is pernicious anaemia (identified by intrinsic factor antibodies), which is a risk factor for developing gastric carcinoma, and should be excluded. Additionally, recent evidence has suggested that some patients have been continued on B12 injections with no clear clinical indication. Recently,guidelines were produced to improve the investigation and management of B12 deficiency. Hence, this audit studied the investigation and management of B12 deficiency and adherence to clinical guidelines in a general practice (GP) in north-west England.Aim: To evaluate the appropriate diagnosis and management of vitamin B12 deficiency in our primary care centre against recognized standards.Methods: Clinical data of patients currently on oral cyanocobalamin and/or intramuscular hydroxocobalamin injections over a 1-year period were audited.Results: Thirty-eight patients (66% females, 34% males) receiving treatment for B12 deficiency were identified. Of these, 55% (21/38) had intrinsic factor antibodies checked and 52% (13/25) were managed according to the guidelines. 100% (8/8) of patients with dietary B12 deficiency (non-vegans) and 75% (3/4) of B12-deficient patients on long-term metformin have had follow-up serum B12 monitoring.Discussion: There is a need to improve the investigation for B12 deficiency, adherence to clinical guidelines, and documentation of patients’ diagnoses, treatment plans, dietary statuses, and required monitoring. We anticipate that adhering to guidelines when appropriate, with clear documentation, will improve the diagnosis and management of vitamin B12 deficiency so that safe prescribing and potential cost savings can be achieved

The Management of Gout in Primary Care - Are We Doing it Right?

Introduction: The soaring incidence of gout in the United Kingdom suggests that medical practitioners should be increasingly aware of optimizing management of gout, which is aimed at pain relief, preservation of joint function and preventing recurrent attacks. Recent guidelines published by the British Society of Rheumatology (BSR), have provided clinicians with a framework for achieving these aims. Aims: To evaluate the management of gout in a primary care centre in North West England against recognized standards. Methods: An electronic search on EMIS Web using the Read codes “gout” and “gouty arthritis”, with a specified period of 2010–2013, generated a cohort of patients who were categorized into 2 groups: those prescribed urate-lowering therapy (allopurinol) and those not. Patients on febuxostat were excluded. Clinical data from the patients were extracted and retrospectively audited. Results: A total of 112 patients were identified, of which only 46% (n = 52) of patients were reviewed after an acute attack. Among those who were prescribed allopurinol, only 19% (n = 12) achieved target serum urate levels while only 67% (n = 42) had their serum urate levels checked regularly and 31% (n = 20) had dosage adjustments. Comparatively, in patients not prescribed allopurinol, a few indications for initiating allopurinol were detected: 29% (n = 12) had more than one attack of gout in a year, 27% (n = 11) suffered from renal insufficiency, 2% (n = 1) presented with tophi, and 17% (n = 7) were on diuretics.Conclusion: Gout management in this primary care centre is not fully concordant to the BSR guidelines. Clearly, there is a need to improve adherence, particularly in the tight monitoring of serum uric acid levels, medications review, appropriate use of allopurinol, where indicated, and patient follow-up

RETROSPECTIVE REVIEW OF SYNACTHEN TESTING IN INFANTS

BackgroundA subnormal cortisol response (30 min level (C30min)&lt;550 nmol/L) to synthetic adrenocorticotrophic hormone/Synacthen test (SDST) in all infants does not necessarily indicate underlying or persistent hypothalamic–pituitary–adrenal axis pathology.MethodsWe retrospectively evaluated the diagnoses and outcomes in 68 infants who had a SDST at age &lt;6 months from 2011 to 2014.Results29 (43%) infants had a subnormal SDST. Causative pathology was identified in 9/29 (31%). In 20/29 (69%) with no identified pathology, repeat SDST was normal in 18/20 (90%) at median age 0.6 (range 0.1–3.2) years but persistently subnormal in 2. Those with a transient abnormality were more likely to be small for gestational age (P=0.03) and had higher initial SDST C30min (390 nmol/L vs 181 nmol/L, P=0.01) than those with pathology.ConclusionSpecific aetiology can be identified in a third of infants with a subnormal SDST. When the aetiology remains elusive, adrenal function should be reassessed as the problem can be transient.</jats:sec

The neuroendocrine sequelae of paediatric craniopharyngioma:a 40 year meta-data analysis of 185 cases from three UK centres

Objectives The management of paediatric craniopharyngiomas was traditionally complete resection (CR), with better reported tumour control compared to that by partial resection (PR) or limited surgery (LS). The subsequent shift towards hypothalamic sparing, conservative surgery with adjuvant radiotherapy (RT) to any residual tumour aimed at reducing neuroendocrine morbidity, has not been systematically studied. Hence, we reviewed the sequelae of differing management strategies in paediatric craniopharyngioma across three UK tertiary centres over four decades. Methods Meta-data was retrospectively reviewed over two periods before (1973–2000 (Group A: n = 100)) and after (1998–2011 (Group B: n = 85)) the introduction of the conservative strategy at each centre. Results Patients had CR (A: 34% and B: 19%), PR (A: 48% and B: 46%) or LS (A: 16% and B: 34%), with trends reflecting the change in surgical approach over time. Overall recurrence rates between the two periods did not change (A: 38% vs B: 32%). More patients received RT in B than A, but recurrence rates were similar: for A, 28% patients received RT with 9 recurrences (32%); for B, 62% received RT with 14 recurrences (26%). However, rates of diabetes insipidus (P = 0.04), gonadotrophin deficiency (P &lt; 0.001) and panhypopituitarism (P = 0.001) were lower in B than those in A. In contrast, post-operative obesity (BMI SDS &gt;+2.0) (P = 0.4) and hypothalamic (P = 0.1) and visual (P = 0.3) morbidity rates were unchanged. Conclusion The shift towards more conservative surgery has reduced the prevalence of hormone deficiencies, including diabetes insipidus, which can be life threatening. However, it has not been associated with reduced hypothalamic and visual morbidities, which remain a significant challenge. More effective targeted therapies are necessary to improve outcomes. </jats:sec