Long-term outcome in juvenile-onset mixed connective tissue disease: a nationwide Norwegian study.

OBJECTIVES: To describe the characteristics, outcome and predictive factors of juvenile mixed connective tissue disease (JMCTD) in a nationwide cohort of patients. METHODS: We examined 55 patients with JMCTD after a mean disease duration of 16.2 years (SD 10.0). Patients were registered according to Kasukawa's criteria. Remission criteria were defined according to those for juvenile idiopathic arthritis, plus absence of cytopenia, myositis, progressive sclerodactyly, lung and oesophageal manifestations. Organ damage was assessed with the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index and the Juvenile Arthritis Damage Index (JADI). Medical records were reviewed for early predictors for outcome, which were assessed by multivariate logistic regression analyses. RESULTS: Three patients developed systemic lupus erythematosus (SLE). Fifty-two patients had continuous JMCTD; the most common manifestations were: Raynaud (100%), arthritis (94%), puffy hands (77%) and pulmonary manifestations (58%). SLE-like, systemic sclerosis (SSc)-like and polymyositis (PM)-like findings were found in 98%, 77% and 48%, respectively. Over time, SLE-like and PM-like manifestations decreased, and SSc-like findings increased. At follow-up, 35 patients (67%) had active disease and 17 (33%) were in remission. In 34 patients (65%), SLICC or JADI≥1 assessments indicated organ damage. Active disease was associated with higher anti-ribonucleoprotein antibody titres at follow-up and positive rheumatoid factor (RF) at diagnosis and follow-up. CONCLUSIONS: Most patients with JMCTD had active disease and organ damage after a mean follow-up of 16.2 years. Active disease was associated with higher anti-ribonucleoprotein antibody levels and positive RF. The presence of RF at diagnosis predicted persistent disease activity.

Diagnostic value of color Doppler ultrasonography of temporal arteries and large vessels in giant cell arteritis: a consecutive case series.

OBJECTIVE: Color Doppler ultrasonography (CDUS) can detect inflammation in the vessel wall. No studies have evaluated the examination of the common carotid artery by CDUS in the diagnostics of giant cell arteritis (GCA). Our aim was to evaluate the combination of CDUS examination of the temporal, axillary, and common carotid arteries in the diagnosis of GCA. METHODS: Patients ages ≥50 years who were referred to our department between April 2010 and October 2012 and suspected to have GCA were consecutively examined. A positive clinical evaluation for GCA 6 months after the first evaluation by 3 rheumatologists was considered as the gold diagnostic standard. All patients underwent CDUS of the temporal, axillary, and common carotid arteries. A biopsy of the temporal artery was performed for most patients. RESULTS: A total of 88 patients were assessed. Forty-six patients were diagnosed to have GCA by the defined gold standard. Forty-eight patients had a positive CDUS of the temporal artery. Forty-six patients diagnosed with GCA had a positive CDUS of the temporal, common carotid, and axillary arteries (100% sensitivity) and 4 patients had a positive CDUS without having GCA (91% specificity). Among the 39 GCA patients that underwent a biopsy, vasculitis was observed in 26 patients (66%), yielding a sensitivity of 67% and a specificity of 95%. CONCLUSION: CDUS of the common carotid, axillary, and temporal arteries had an excellent sensitivity and high specificity to diagnose GCA. CDUS has the potential to replace biopsy in ordinary clinical care without compromising on sensitivity and specificity.

Leflunomide as a corticosteroid-sparing agent in giant cell arteritis and polymyalgia rheumatica: a case series.

OBJECTIVES: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) affect individuals older than 50 years of age and corticosteroids are the mainstay of treatment. The aim of our study was to explore the role of leflunomide as a corticosteroid-sparing agent in GCA and PMR patients. METHODS: Patients with difficult-to-treat GCA and PMR were retrospectively identified in the period from 2010 to 2013. The doses of corticosteroids and CRP values were noted before, after three months, and at the end of the treatment with leflunomide (for patients continuing treatment, censoring date was January 1, 2013). RESULTS: Twenty-three patients were identified (12 with PMR and 11 with GCA). A reduction of 6 mg/dL (CI 95% -10.9-34.2, P = 0.05) in CRP and 3.7 mg (CI 95% 0.5-7.0, P = 0.03) in prednisolone dose was observed in the PMR group. In GCA patients, the reduction was 12.4 mg/dL (CI 95% 0.7-25.5, P = 0.06) in CRP and 6.6 mg (CI 95% 2.8-10.3, P < 0.01) in prednisolone dose. CONCLUSION: Leflunomide seems to be effective as a corticosteroid-sparing agent in patients with difficult-to-treat GCA and PMR. Randomized controlled trials are warranted in order to confirm the usefulness of leflunomide in the therapy of GCA/PMR.

Interleukin 1ß blockade improves signs and symptoms of chronic calcium pyrophosphate crystal arthritis resistant to treatment.

A 54-year-old woman with recurrent episodes of bilateral knee arthritis was admitted several times to the Department of Rheumatology. She was diagnosed to have chronic calcium pyrophosphate crystal arthritis. Interleukin 1ß (IL-1ß) plays a central role in the pathogenesis of inflammation induced by calcium pyrophosphate crystals, and IL-1ß blockade may be an effective treatment in patients with severe chronic calcium pyrophosphate crystal arthritis. Because of the short effect of treatment with corticosteroids and the frequent attacks of arthritis, the patient was treated with the IL-1 receptor antagonist anakinra. She responded well after 1 week with normalization of inflammation. Eight months after the initiation of treatment, the patient has had no relapses, although we were unable to withdraw the anakinra.

Severe deterioration of newly diagnosed Takayasu arteritis in a patient re-treated with interferon beta-1α for concomitant longstanding multiple sclerosis.

A 52-year-old woman with Takayasu arteritis and a known history of multiple sclerosis had been treated with subcutaneous interferon (IFN) beta-1α. After the re-introduction of the IFN beta-1α, the patient had a gradual worsening of the arteritis, with claudication symptoms in the left arm and increased inflammation markers. An evaluation using Doppler ultrasound of the supra-aortic vessels revealed severe stenosis of the left axillary artery. The IFN beta-1α was withdrawn, with prompt clinical and laboratory improvement of the vasculitis.