A systematic review and meta-analysis to inform cancer screening guidelines in idiopathic inflammatory myopathies.

OBJECTIVES: To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to cancer screening. METHODS: A systematic literature search was carried out on Medline, Embase and Scopus. Cancer risk within the IIM population (i.e. not compared with the general population) was expressed as risk ratios (RR) for binary variables and weighted mean differences (WMD) for continuous variables. Evidence relating to cancer screening practices in the IIMs were synthesized via narrative review. RESULTS: Sixty-nine studies were included in the meta-analysis. DM subtype (RR 2.21), older age (WMD 11.19), male sex (RR 1.53), dysphagia (RR 2.09), cutaneous ulceration (RR 2.73) and anti-transcriptional intermediary factor-1 gamma positivity (RR 4.66) were identified as being associated with significantly increased risk of cancer. PM (RR 0.49) and clinically amyopathic DM (RR 0.44) subtypes, Raynaud's phenomenon (RR 0.61), interstitial lung disease (RR 0.49), very high serum creatine kinase (WMD -1189.96) or lactate dehydrogenase (WMD -336.52) levels, and anti-Jo1 (RR 0.45) or anti-EJ (RR 0.17) positivity were identified as being associated with significantly reduced risk of cancer. Nine studies relating to IIM-specific cancer screening were included. CT scanning of the thorax, abdomen and pelvis appeared to be effective in identifying underlying asymptomatic cancers. CONCLUSION: Cancer risk factors should be evaluated in patients with IIM for risk stratification. Screening evidence is limited but CT scanning could be useful. Prospective studies and consensus guidelines are needed to establish cancer screening strategies in IIM patients.

Methotrexate in the Treatment of Idiopathic Granulomatous Mastitis.

OBJECTIVE: Idiopathic granulomatous mastitis (IGM) is a disfiguring inflammatory breast disease without effective treatment. We report the largest IGM cohort treated with methotrexate (MTX) monotherapy. METHODS: Chart review was performed on patients evaluated by the Stanford Immunology and Rheumatology Clinic, with histopathologically established IGM treated with MTX, and at least 1 followup appointment. RESULTS: Nineteen female patients with a mean age of 33.5 years were identified. Most failed treatment with antibiotics, prednisone, and surgical intervention. By 15 months of treatment with MTX, 94% had disease improvement and 75% achieved disease remission. CONCLUSION: MTX monotherapy is an effective treatment for IGM.

A Patient with Sjogren's Syndrome and Subsequent Diagnosis of Inclusion Body Myositis and Light-Chain Amyloidosis.

We discuss a challenging case of a 58-year-old Vietnamese-American woman who presented to her new primary care provider with an 8-year history of slowly progressive dysphagia, hoarseness, muscle weakness with associated frequent falls, and weight loss. She eventually reported dry eyes and dry mouth, and she was diagnosed with Sjogren's syndrome. Subsequently, she was additionally diagnosed with inclusion body myositis and gastric light-chain (AL) amyloidosis. Although inclusion body myositis has been previously associated with Sjogren's syndrome, inclusion body myositis is rare in non-Caucasians, and the trio of Sjogren's syndrome, inclusion body myositis, and AL amyloidosis has not been previously reported. Sjogren's syndrome is a systemic autoimmune condition characterized by ocular and oral dryness. It is one of the most common rheumatologic disorders in the USA and worldwide. Early diagnosis of Sjogren's is particularly important given the frequency and variety of associated autoimmune diseases and extraglandular manifestations. Furthermore, although inclusion body myositis has a low prevalence, it is the most common inflammatory myopathy in older adults and is unfortunately associated with long delays in diagnosis, so knowledge of this disorder is also crucial for practicing internists.

Anti-MDA5-Positive Dermatomyositis Presenting as Fever of Unknown Origin.

Dermatomyositis is a chronic systemic autoimmune disease characterized by inflammatory infiltrates in the skin and muscle. The wide variability in clinical and serologic presentation poses a diagnostic challenge for the internist. Appreciation of the clinical variants of dermatomyositis allows for expedient diagnosis and avoidance of diagnostic error. We illustrate these challenges with the case of a 51-year-old Vietnamese-American man who initially presented with fever of unknown origin in the absence of overt skin and muscle manifestations. The diagnosis of dermatomyositis was not evident on several clinical encounters due to the absence of these hallmark symptoms. We review the variable clinical manifestations of a subtype of dermatomyositis associated with an autoantibody against melanoma differentiation-associated protein 5 (anti-MDA5) and suggest consideration of dermatomyositis as a diagnosis in patients presenting with systemic illness and markedly elevated ferritin, even in the absence of elevated muscle enzymes and classic autoantibodies.

Corticosteroids in Myositis and Scleroderma.

Idiopathic inflammatory myopathies (IIMs) involve inflammation of the muscles and are classified by the patterns of presentation and immunohistopathologic features on skin and muscle biopsy into 4 categories: dermatomyositis, polymyositis, inclusion body myositis, and immune-mediated necrotizing myopathy. Systemic corticosteroid (CS) treatment is the standard of care for IIM with muscle and organ involvement. The extracutaneous features of systemic sclerosis are frequently treated with CS; however, high doses have been associated with scleroderma renal crisis in high-risk patients. Although CS can be effective first-line agents, their significant side effect profile encourages concomitant treatment with other immunosuppressive medications to enable timely tapering.

Quality of life before and after cosmetic surgery.

This article reviews the literature regarding the impact of cosmetic surgery on health-related quality of life (QOL). Studies were identified through PubMed/Medline and PsycINFO searches from January 1960 to December 2011. Twenty-eight studies were included in this review, according to specific selection criteria. The procedures and tools employed in cosmetic surgery research studies were remarkably diverse, thus yielding difficulties with data analysis. However, data indicate that individuals undergoing cosmetic surgery began with lower values on aspects of QOL than control subjects, and experienced significant QOL improvement post-procedurally, an effect that appeared to plateau with time. Despite the complexity of measuring QOL in cosmetic surgery patients, most studies showed an improvement in QOL after cosmetic surgery procedures. However, this finding was clouded by measurement precision as well as heterogeneity of procedures and study populations. Future research needs to focus on refining measurement techniques, including developing cosmetic surgery-specific QOL measures.

Evidence-based, non-surgical treatments for vitiligo: a review.

Vitiligo is an acquired pigmentary disorder characterized by depigmented macules and patches secondary to the loss of functional melanocytes. It is a chronic disease that affects between 0.1% and 2% of the general population, affecting both sexes and all races. The appearance and the unpredictable course are psychologically and socially devastating. The success of current therapeutic options is limited. The objective of this review was to assess non-surgical treatments of vitiligo and to determine if comparing these studies can lead to (1) practical applications in the clinical setting and (2) recommendations for future research including study design and topics to be investigated further. Combination therapies were found to be more effective than monotherapy, and most combinations included a form of phototherapy, of which narrow-band-UVB was found to be most effective with the least adverse effects. Topical treatment with corticosteroids, immunomodulators, vitamin D analogs, and psoralens had mixed outcomes. Oral therapies including antioxidants were helpful adjuvants to treatment. Studies lacked consistent design, mechanism of disease assessment, and long-term follow-up. Sample size was also frequently limited. This review found that while several non-surgical therapies exist for the treatment of vitiligo, their usefulness, especially in the long term, is not well understood. Those studies that were able to elicit repigmentation often lacked an assessment on quality of life and/or patient satisfaction. More standardized methods of study design and assessment are needed to compare outcomes and make definitive conclusions on treatment effectiveness.