SAPHIRE: Stress and Pulmonary Hypertension in Rheumatoid Evaluation-A Prevalence Study.

Pulmonary artery hypertension (PAH) is a disorder of elevated resistance in the pulmonary arterial vessels, reflected by elevation of measured pulmonary artery pressure (PAP), and presenting with breathlessness and, if untreated, progressing to right heart failure and death. The heightened prevalence of PAH in populations with underlying systemic autoimmune conditions, particularly scleroderma and its variants, is well recognised, consistent with the proposed autoimmune contribution to PAH pathogenesis, along with disordered thrombotic, inflammatory, and mitogenic factors. Rheumatoid arthritis (RA) is one of a group of systemic autoimmune conditions featuring inflammatory symmetrical erosive polyarthropathy as its hallmark. This study explored the prevalence of PAH in a population of unselected individuals with RA, using exercise echocardiography (EchoCG). The high prevalence of EchoCG-derived elevation of PAP (EDEPP) in this population (14%) suggests that, like other autoimmune conditions, RA may be a risk factor for PAH. Patients with RA may therefore represent another population for whom PAH screening with noninvasive tools such as EchoCG may be justified.

The natural history of interferon-alpha2b-induced thyroiditis and its exclusivity in a cohort of patients with chronic hepatitis C infection.

BACKGROUND: Interferon-alpha2b (IFN-alpha2b) is well known to cause both hyper- and hypo-thyroidism. In the former, the commonest aetiology is thyroiditis. As there is no previous data to fully characterize the entity of IFN-related thyroiditis, the aim of this study is to document in detail its evolution in a cohort of hepatitis C patients treated with pegylated IFN-alpha2b and Ribavirin (RBV). METHODS: A prospective observational study was conducted in patients who developed thyroid diseases whilst receiving combination of pegylated IFN-alpha2b and RBV for hepatitis C. The patients were followed with monthly thyrotropin (TSH). Where TSH was undetectable, free tetra- (fT4) and tri-iodothyronine (fT3) were added. Anti-thyroperoxidase (TPO), anti-thyroglobulin (Tg) and thyroid stimulating immunoglobulin (TSI) levels were also performed at diagnosis, during and at the end of IFN therapy. All patients were assessed and followed up closely with monthly TSH, fT4 and fT3 levels until the completion, after 6 and 12 months of treatment. RESULTS: There were seven females and four males over a 30-month period. All patients were found to have thyroiditis. On average, the time to the development of thyroid disease was 10 weeks and duration of disease 9 weeks. All patients eventually recovered normal biochemical thyroid function although two required short-term supplementation. CONCLUSION: Thyroiditis was found exclusively in our patients. Both the hyper- and hypo-thyroid phase can be short lived, extreme and transient in nature which warrants strict monthly TSH monitoring. Careful follow-up of all patients is mandatory as complete recovery is expected.

Update on the immunology, diagnosis and management of systemic lupus erythematosus.

Lupus occurs with a prevalence of 2-9 in 10,000 people, targeting female and indigenous populations in particular. Lupus and related systemic autoimmune syndromes (scleroderma, Sjögren's syndrome, rheumatoid arthritis and polymyositis) result from a similar set of genetically and environmentally modulated immune disturbances, and the diagnostic and management approach to these conditions is broadly comparable. Evanescent, vague symptoms, restrictive diagnostic criteria and low diagnostic suspicion might have resulted in the under-diagnosis of these problems in the past, imposing considerable burdens on sufferers and the community. Serological screening should be employed cautiously and wisely, given the rapidly occurring changes in methodology, which have lowered their specificity. Close liaison with the immunology laboratories performing these tests is therefore advisable. Clinicians should emphasize the improving prognosis of lupus and related conditions as a result of earlier disease detection, improved educational support systems and refined medical therapies.

Impact of hydroxychloroquine therapy on chronic urticaria: chronic autoimmune urticaria study and evaluation.

BACKGROUND: Chronic urticaria (CU) imposes profound impairment on quality of life. Up to 60% of idiopathic CU is associated with autoimmune phenomena, and may respond to immunomodulation. Hydroxychloroquine offers potential efficacy for CU and is relatively benign compared with most other therapeutic approaches. OBJECTIVE: The aim of the chronic autoimmune urticaria study and evaluation was to evaluate the efficacy of hydroxychloroquine in patients with chronic idiopathic urticaria. METHODS: Twenty-one patients referred to the Immunology and Allergy Unit at John Hunter Hospital, New South Wales, Australia, with idiopathic CU were randomised to receive treatment with standard urticaria therapies (corticosteroids, H2-antihistamines, H1--antihistamines, doxepin) with or without hydroxychloroquine. Markers of autoimmunity, thyroid disease and mast-cell autoreactivity (autologous serum skin-prick testing (ASPT)) were assessed. Measures of urticaria control were compared at baseline and at 12 weeks for the 18 individuals who completed the study. These included urticaria scores, medication scores and quality-of-life indices. RESULTS: The hydroxychloroquine-treated group achieved significant improvements in quality of life as assessed by the global symptom severity score and the LAMY-7 (a quality of life index designed by Lamy, 7th revision) at 12 weeks (P < 0.01 and P < 0.05, respectively). No significant treatment effect on medication requirements or urticaria score was detected, although differences between treatment groups approached statistical significance for urticaria score and medication requirements (0.05 < P < 0.10). ASPT-reactivity did not correlate to hydroxychloroquine-responsiveness. Hydroxychloroquine was well tolerated. CONCLUSION: Immunomodulation with hydroxychloroquine is safe and appears to offer some efficacy as an intervention in CU.