Long-term follow-up of patients with idiopathic inflammatory myopathy at Waitemata District Health Board.

BACKGROUND: Idiopathic inflammatory myopathies (IIMs) are an uncommon group of diseases that can be associated with significant morbidity and mortality related to systemic involvement or treatment-related complications. AIM: This study reports the concomitant diseases, extent of organ involvement, immunosuppressive use, treatment-related complications and damage outcome in a cohort of adult IIM. METHODS: All patients with IIM fulfilling at least 3 of the 4 Bohan and Peter criteria were identified. Medical notes were reviewed retrospectively. Data was collected for clinical presentation, autoantibody profile, immunosuppressives received, treatment-/disease-related complications and mortality rate. Systemic involvement was divided into 12 organ systems. Patient damage index was calculated using the Myositis Damage Index (MDI) tool. RESULTS: Twenty patients were identified from a single centre, with median duration of follow-up of 9 years. The majority of patients had polymyositis, with the remaining having dermatomyositis. Osteoporosis and osteopenia were the most common concomitant diseases observed, at 111 per 1,000 patient years, followed by lung involvement with 78 events per 1,000 patient years follow-up. ANA was positive in 6/20(30%) patients with Jo-1 positivity in 10% of patients. Nineteen patients (95%) received steroids. There were significant steroid-related complications, with 85% of patients developing osteopenia/osteoporosis. The mean MDI score was 3.11. The mortality rate was 52/1,000 patient years and infection was the most common cause of death. CONCLUSION: There is significant treatment-related morbidity in adult IIM, with a high incidence of steroid-related complications, in particular, osteoporosis. Infections accounted for the most common cause of death.

Screening for pulmonary arterial hypertension in patients with scleroderma--a New Zealand perspective.

BACKGROUND: Pulmonary arterial hypertension (PAH) in scleroderma (SSc) patients is a devastating complication with high mortality if untreated. Early recognition and specific treatment of PAH may improve outcome. Regular interval screening for PAH is generally recommended in scleroderma patients especially with the availability of emerging new therapies. The aim of this study is to determine the self-reported screening and treatment practices for SSc-PAH amongst rheumatologists in New Zealand (NZ). METHODS: An anonymous online questionnaire survey was emailed to all rheumatologists in New Zealand. RESULTS: Responses were received from 65% (39/60) of rheumatologists. The majority of patients had limited SSc (lcSSc) (57%) versus diffuse SSc (dcSSc) (34%). Twelve percent of patients had PAH. Eighty-two percent of rheumatologists screened for PAH in all SSc patients regardless of symptoms. The most commonly used screening modalities were pulmonary function tests (PFT) (97%) followed by clinical examination (95%) and echocardiogram (TTE) (92%). The majority of rheumatologists performed screening tests on a yearly basis (80% used PFT and 64% used TTE). A right heart catheter was used to confirm PAH in 70% of patients. Sixty-four percent of rheumatologists extend screening interval time if their patients were clinically stable. The most common PAH-specific therapy used was sildenafil (57%) followed by bosentan (19%). Sixty-four percent of rheumatologists supported a national PAH-SSc screening guideline. CONCLUSION: This study has shown a wide variability of how NZ rheumatologists screen for PAH in scleroderma patients. The development of a PAH-SSc guideline for screening and diagnosis may help standardise treatment practices in NZ.

A retrospective seven-year analysis of the use of B cell depletion therapy in systemic lupus erythematosus at University College London Hospital: the first fifty patients.

OBJECTIVE: To describe the 6-month clinical outcome and the long-term safety profile of B cell depletion therapy (BCDT) in 50 patients with active systemic lupus erythematosus (SLE), who were nonresponsive or poorly responsive to conventional immunosuppression. METHODS: All except 4 of 50 patients with active SLE received 1 gm of rituximab, 750 mg of cyclophosphamide, and 100-250 mg of methylprednisolone, administered on 2 occasions 2 weeks apart, to achieve B cell depletion. Clinical outcome was assessed using the British Isles Lupus Assessment Group (BILAG) activity index and serial serologic measurements of disease activity. Remission was defined as a change from a BILAG A or B score to a C or D score in every organ system. Partial remission was a change from a BILAG A or B score to a C or D score in at least 1 system, but with the persistence of 1 score of A or B in another system. No improvement was defined as a BILAG A or B score that remained unchanged after treatment. RESULTS: Of the 45 patients available for followup at 6 months, 19 patients (42%) achieved remission, and 21 patients (47%) reached partial remission after 1 cycle of BCDT (mean followup 39.6 months). BCDT resulted in a decrease in median global BILAG scores from 12 to 5 (P < 0.0001) and median anti-double-stranded DNA antibody titers from 106 to 42 IU/ml (P < 0.0001), and an increase in the median C3 level from 0.81 to 0.95 mg/liter (P < 0.02) at 6 months. Five serious adverse events were observed. CONCLUSION: BCDT is an effective treatment for patients with active SLE whose disease has failed to respond to standard immunosuppressive therapy. Although the safety profile of BCDT is favorable, ongoing monitoring is required.

Inflammatory muscle disease assessment.

In recent years, idiopathic inflammatory muscle disease assessment has advanced significantly with the development of an international consensus on outcome measurement indices. The International Myositis Assessment and Clinical Studies Group continues to evaluate the validity and reliability of various disease activity and disease damage tools, which will facilitate adequately controlled randomized trials of recently developed therapeutic agents.

Sjögren's syndrome: diagnosis and therapeutic challenges in the elderly.

Sjögren's syndrome (SS) is a chronic autoimmune rheumatic disease characterized by keratoconjunctivitis sicca and xerostomia as a result of lymphocytic infiltration of the lacrimal and salivary glands. Extra-glandular manifestations occur in about one-third of patients with SS. The diagnosis of SS in the geriatric population is not straightforward and consideration needs to be given to exclusion of other conditions that may have similar presenting symptoms. The presence of autoantibodies, in particular anti-Ro and anti-La antibodies, may aid in the diagnosis of SS. Salivary gland biopsy findings represent one of the objective criteria included in the widely accepted American-European classification diagnostic criteria. However, SS-like histological changes can also be present in the healthy elderly, adding to the dilemma in diagnosing this condition in the geriatric population. Management of SS involves local treatment of dry eyes and mouth with replacement and stimulation therapies. Patients with more serious systemic involvement may require immunosuppressive therapy. Medications that are routinely used in the treatment of patients with SS often have limited use in the elderly population because patients in the latter group may have complex co-morbid conditions and be taking multiple medications. Recently, use of newer targeted therapies has been explored in SS. This article provides an update on recent developments in the diagnosis and management of SS, with emphasis on issues that may arise when treating elderly patients with this condition.

B cell depletion therapy in systemic lupus erythematosus: long-term follow-up and predictors of response.

OBJECTIVES: To describe the long-term clinical outcome and safety profile of B cell depletion therapy (BCDT) in patients with systemic lupus erythematosus (SLE). It was also determined whether baseline parameters can predict the likelihood of disease flare. METHODS: 32 patients with refractory SLE were treated with BCDT using a combination protocol (rituximab and cyclo-phosphamide). Patients were assessed with the British Isles Lupus Assessment Group (BILAG) activity index, and baseline serology was measured. Flare was defined as a new BILAG 'A' or two new subsequent 'B's in any organ system. RESULTS: Of the 32 patients, 12 have remained well after one cycle of BCDT (median follow-up 39 months). BCDT was followed by a decrease of median global BILAG scores from 13 to 5 at 6 months (p = 0.006). Baseline anti-extractable nuclear antigen (ENA) was the only identified independent predictor of flare post-BCDT (p = 0.034, odds ratio = 8, 95% CI 1.2 to 55) from multivariable analysis. Patients with low baseline serum C3 had a shorter time to flare post-BCDT (p = 0.008). Four serious adverse events were observed. CONCLUSION: Autoantibody profiling may help identify patients who will have a more sustained response. Although the long-term safety profile of BCDT is favourable, ongoing vigilance is recommended.