The Darker Facets of Systemic Lupus Erythematosus (SLE).

Cerebrovascular events remain a rare but serious feature of systemic lupus erythematosus (SLE). In this report, we see a 25-year-old lady who presented with sudden-onset right-sided weakness and speech disturbances. She was initiated on anti-platelet therapy and glucocorticoids. Her admission was complicated by worsening kidney function due to lupus nephritis. She responded well to immunosuppressant therapy and was discharged following resolution of her symptoms for outpatient specialist follow-up. The rarity of such cases poses a diagnostic and treatment challenge. A language barrier and difficult social circumstances can exacerbate this. However, awareness of neuropsychiatric lupus as a differential diagnosis at the acute assessment of stroke and early involvement of specialist teams, allied health professionals, and safeguarding teams can lead to a successful long-term outcome.

Rheumatoid arthritis: previously untreated early disease.

INTRODUCTION: Rheumatoid arthritis is a chronic autoimmune disease, which most often presents as a symmetrical polyarthritis of the hands and feet. Pharmacological treatments include non-steroidal anti-inflammatory drugs (NSAIDs), glucocorticoids (GCs) and other disease-modifying anti-rheumatoid drugs (DMARDs), which may be synthetic (either conventional [csDMARDs] or targeted [tsDMARDs]) or biological (bDMARDs). METHODS AND OUTCOMES: We conducted a systematic overview, aiming to answer the following clinical questions: What are the effects of methotrexate in combination with other csDMARDs versus methotrexate monotherapy in people with rheumatoid arthritis who have not previously received any DMARD treatment (first-line treatment)? What are the effects of bDMARDs as monotherapy versus methotrexate or other csDMARDs in people with rheumatoid arthritis who have not previously received any DMARD treatment (first-line treatment)? What are the effects of bDMARDs in combination with methotrexate versus methotrexate monotherapy or other csDMARDs in people with rheumatoid arthritis who have not previously received any DMARD treatment (first-line treatment)? What are the effects of glucocorticoids in combination with methotrexate or with other csDMARDs versus methotrexate or other csDMARDs in people with rheumatoid arthritis who have not previously received any DMARD treatment (first-line treatment)? We searched: Medline, Embase, The Cochrane Library and other important databases up to December 2014 (Clinical Evidence overviews are updated periodically; please check our website for the most up-to-date version of this overview). RESULTS: At this update, searching of electronic databases retrieved 2058 studies. Of the full articles evaluated, 10 systematic reviews, 22 RCTs, and one follow-up report were added at this update. We performed a GRADE evaluation for 18 PICO combinations. CONCLUSIONS: In this systematic overview, we categorised the efficacy for 22 comparisons based on information about the effectiveness and safety of bDMARDs (monotherapy or combined with csDMARDs), csDMARDs (monotherapy or combined with other csDMARDs), glucocorticoids combined with methotrexate or other csDMARDs, and methotrexate (monotherapy or combined with other csDMARDs), identifying interventions which were likely or unlikely to be beneficial.

Rare association of antisynthetase syndrome and Kennedy's disease.

Antisynthetase syndrome is a type of Idiopathic Inflammatory Myopathy (IIM) associated with anti-Jo1 antibody. Kennedy's disease or X-linked spinal and bulbar muscular atrophy (SBMA) is a rare neuromuscular disease. We describe the case report of a 53-year-old man who presented with proximal muscle weakness and a history of bilateral hand tremor. Initial physical examination demonstrated "mechanic's hands", Raynaud's phenomenon, having elevated creatine kinase and lactate dehydrogenase levels and anti-Jo1 antibody positivity. His muscle biopsy demonstrated inflammatory infiltrate characteristic of IIM. Considering these findings, we reached the diagnosis of antisynthetase syndrome and commenced immunosuppressive therapy. On follow-up examination, he had developed dysphagia, and his tremor had worsened. His electroneurogram result was characteristic of Kennedy's disease, and the genetic test result showed an allele with 44 CAG repeat expansion in the androgen receptor gene of the X chromosome. This confirmed that in addition to antisynthetase syndrome, he also had Kennedy's disease. This patient now receives immunology and neurology follow-up. His symptoms have improved with low dose corticosteroids, propranolol for tremor, vitamin B supplementation, and physiotherapy. This article presents a rare case report of a patient with concurrent antisynthetase syndrome and Kennedy's disease, both of which lead to elevated creatine kinase levels and muscle weakness, thus, underpinning the importance of careful follow-up of patients with IIM and maintaining an open mind to other diagnoses when faced with refractory and/or new symptoms.