So last night I couldn't sleep... I had a slight fever. Found some articles regarding case studies and my disease... thought I would share. Hope everyone is enjoying the nice weather!
Ten-Year Survival Poor in Antisynthetase Syndrome
By: NANCY WALSH
BARCELONA — A review of 30 patients with antisynthetase syndrome found that only half survived 10 years after diagnosis, Dr. Oyvind Palm reported at the annual European Congress of Rheumatology.
This idiopathic inflammatory myopathy is characterized by the presence of antibodies directed against tRNA synthetase. The most common antibody is anti-Jo-1, which is found in 80% of cases.
Other antibodies sometimes found include anti-SSA, anti-PL-7, and anti-PL-12.
Clinical manifestations of the disease include interstitial lung disease, which can be severe, arthritis, Raynaud phenomenon, and the hyperkeratotic rash known as mechanic's hands, according to Dr. Palm of the department of rheumatology, Rikshospitalet-Radiumhospitalet Medical Center, Oslo.
With the aim of characterizing the disease's clinical and serologic features, researchers reviewed all hospital records of patients diagnosed with an inflammatory myopathy and analyzed the charts of those who had antisynthetase antibodies and pulmonary disease.
The mean age of these 30 patients was 45.5 years, and in one-third of the group, the disease onset was before age 40. Two-thirds of the patients were women.
Most patients had histologic evidence of inflammatory myopathy and elevated serum creatine kinase, but only four had elevations of creatine kinase exceeding 3,000 IU/mL.
Muscular manifestations rarely caused significant patient disability and were present at the onset of disease in only six of the cases.
Anti-Jo-1 antibodies were detected in 90%. Anti-SSA autoantibodies, commonly found in patients with Sjögren syndrome, were detected in 50% but only rarely were they associated with dry eyes and mouth, Dr. Palm wrote in a poster session.
Pulmonary involvement was classified as follows:
▸ Type I (acute): Found in 24%; rapid onset of dyspnea or cough with development of hypoxemia within 1 month after the onset of disease.
▸ Type II (subacute): Found in 64%; gradual onset of pulmonary symptoms.
▸ Type III (asymptomatic): Found in 12%; coincidentally detected pulmonary abnormalities on x-ray or CT scan with subsequent slowly developing pulmonary symptoms.
Honeycombing with end-stage pulmonary disease was found in 30.4%.
All but one patient had received treatment with immunosuppressive drugs including corticosteroids, cyclophosphamide, and rituximab.
Four patients died, two having type I pulmonary involvement. "While approximately 90% survive the first 3 years of disease, thereafter the mortality increases sharply, and new treatment strategies are clearly warranted," he concluded.
B-Cell Depletion With Rituximab Appears Promising for Myopathies
BARCELONA — Clinical improvement in a small series of patients with inflammatory myopathies treated with rituximab suggests that B-cell depletion may prove useful in these disorders, according to Dr. Marlies Blom of the department of rheumatology, Radboud University Nijmegen (the Netherlands) Medical Centre.
Among seven patients with dermatomyositis, polymyositis, or antisynthetase syndrome, two infusions of 1,000 mg rituximab 2 weeks apart resulted in a mean 30% increase in muscle strength at 3 months, Dr. Blom reported in a poster session at the annual European Congress of Rheumatology.
Patients' subjective reports of improvement in muscle strength were confirmed by handheld dynamometry.
The patients ranged in age from 38 to 58 years, and the duration of their disease ranged from 3 to 16 years. Four of the seven were female.
Previous treatments included oral and intravenous prednisone, methotrexate, azathioprine, cyclophosphamide, interferon, etanercept, and intravenous immunoglobulin.
A mean 13% improvement was reported on Health Assessment Questionnaire (HAQ) scores, and improvements also were seen in levels of creatine phosphokinase, a marker of disease activity.
In one patient, a muscle biopsy taken 4 months after treatment showed a total absence of CD20+ B cells. This patient's Disease Activity Score-28 (DAS28) score fell from 6.8 to 4.5 after 3 months, according to Dr. Blom.
After initial good response, three patients required retreatment for exacerbations of myositis at about 6 months.
No serious adverse events were observed and immunoglobulin levels remained within normal levels.
These results suggest that B cells play an important role in the pathogenesis of inflammatory myopathies, Dr. Blom noted.
Another recent report suggested that a possible rationale for considering B-cell depletion as a therapeutic strategy in dermatomyositis was that treatment with rituximab had previously been shown to result in improvements in muscle strength in humorally mediated autoimmune peripheral neuropathies (Arthritis Rheum. 2005;52:601-7).
The importance of humoral immunity in dermatomyositis also is suggested by the observation that perifascicular endothelial immunoglobulin and complement deposition are thought to result in the muscle ischemia and atrophy (J. Rheumatol. 2006;33:1021-6).
Furthermore, the observation that there are antibodies specific for myositis also supports the concept of B-cell-mediated humoral abnormality in dermatomyositis (Medicine [Baltimore] 1991;70:360-74).