Despite their disadvantages, glucocorticoids (GCs) remain a mainstay of therapy for polymyalgia rheumatica (PMR). Second-line antirheumatic and immune-modulatory drugs are not infrequently required because of disease relapses during GC tapering and GC adverse effects. Therapy with methotrexate or with an anti-tumor necrosis factor drug showed modest efficacy in this situation. Tocilizumab (TCZ) is an anti-interleukin 6 receptor antibody that is being recently studied in the treatment of PMR patients who are intolerant or refractory to GCs, especially after failure of a second-line agent. We report a case of PMR in which GCs were stopped because of adverse effects despite good response. The condition responded to neither methotrexate nor etanercept. Treatment with TCZ has led to significant improvement of the patient's clinical and biochemical PMR activity parameters, and she was kept in a solid remission for 1 year without any TCZ-related adverse effects. Tocilizumab is a promising drug in the management of PMR. Further studies are required to clearly define the indications and duration of TCZ therapy in the management of PMR.
Diffuse alveolar hemorrhage (DAH) is a life-threatening complication of systemic lupus erythematosus (SLE). Cases complicated with DAH often have active SLE with multi-organ involvement, especially lupus nephritis. We describe a rare case of DAH as the first presenting manifestation of SLE in the absence of lupus nephritis. Remission was induced by IV methylprednisolone, IV cyclophosphamide, and plasmapheresis. Further cycles of cyclophosphamide were prevented by recurrent infections. Maintenance of remission was successfully achieved with oral mycophenolate mofetil 1 g twice daily, with a good control of SLE and without further DAH episodes.
Rheumatologists are increasingly aware of the entity synovitis with pitting edema. The remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome has been reported with an array of conditions that include polymyalgia rheumatica, rheumatoid arthritis, Sjögren's syndrome and psoriatic arthropathy. Synovitis with pitting edema is now being increasingly recognized with systemic lupus erythematosus (SLE). We report a patient who presented with edema of hands and feet and was diagnosed eventually with definite SLE. With magnetic resonance imaging, joint effusions and tenosynovitis were confirmed to be associated with the otherwise-unexplained extremity edema.
Background
The prevalence of primary headache in patients with systemic lupus erythematosus (SLE) varies widely and whether it should be attributed to neurological involvement is controversial. We aimed to investigate the prevalence and characteristics of headache in SLE patients, describe its association with disease-related variables and brain imaging, and explore its impact on life.
Results
The overall prevalence of headache was 54.4%, and migraine was the most common type among headache sufferers (48.4%). Headache severity (VAS) and impact (HIT-6) correlated with SLEDAI-2K (P = 0.019 and P < 0.001, respectively) as well as with each other (P = 0.006). Brain imaging abnormalities were found in 25.8%, with white-matter hyperintensities (WMH) being the most frequent pathology. Musculoskeletal manifestations, positive anti-phospholipid (aPL) antibodies, and SLEDAI score ≥ 13.5 were identified as predictors of headache.
Conclusion
Primary headache, especially migraine, is a common feature of patients with SLE, and its presence is associated with negative impact on quality of life. Musculoskeletal features, aPL positivity, and overall disease activity appear to predict primary headache in SLE.
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