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Involvement of cardiac muscle is felt to be very uncommon in anti-HMGCR myopathy, and therefore early cardiac evaluation is not considered a high priority for this condition. We herein present the case of a 72 year-old woman admitted due to dyspnea and orthopnea, who, in retrospect, suffered from proximal more than distal muscle weakness for 3 months prior to admission. She was found to have acute systolic heart failure. Serologic testing showed positive 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) IgG antibodies, and muscle biopsy showed necrotizing myopathy. No alternative explanation for heart failure was found. Despite immunotherapy and symptomatic treatment, she died from multiorgan failure. Our study suggests that heart failure in anti HMGCR myopathy may not be as rare as previously thought, and therefore early cardiac evaluation should be considered in patients with this diagnosis, to minimize morbidity and mortality.

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OBJECTIVES: This study was aimed to investigate the significance of unexpected vasculitis identified in gastrointestinal (GI) specimens by determining its prevalence and correlation with clinical outcomes. METHODS: GI specimens with histologic evidence of vasculitis were identified in our pathology database over a 10-year period (January 2008 to August 2018). Clinical history, treatment, and follow-up were reviewed. RESULTS: Of the 131,367 GI pathology cases received over the 10-year study period, 29 (0.02%) cases showed histologic evidence of GI vasculitis. The majority (69%, 20/29) were not clinically suspected. Of these, 20% (4/20) of patients were subsequently diagnosed with systemic vasculitis. During the mean follow-up period of 34.0 months, 24% (4/17) of the patients with this unexpected diagnosis died as the result of direct complications of GI vasculitis. We also found that 95% of cases with unexpected vasculitis in their GI pathology specimens were communicated in a timely manner to the ordering physicians, which necessitated the immediate initiation of additional workups in 85% of these patients. CONCLUSIONS: The GI involvement of vasculitis is rarely encountered by pathologists, but its diagnosis carries tremendous clinical significance with a high mortality rate. Therefore, timely communication is highly recommended for the early diagnosis and treatment of this disease.

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In previous editorials, Chapman and Otis in 2011 and Layfield in 2014 have summarized much of the work responsible for establishing the concept of critical diagnoses in surgical pathology and cytopathology. Both editorials end with a list of 8 key policy points needed for an effective strategy of handling and communicating critical diagnoses. We have developed and distributed a Web-based survey to elicit clinicians' attitudes regarding many of those key policy points, such as how, when, and to whom critical diagnoses should be reported; we have allowed some level of collaboration with the clinical staff when developing our communication policies as the Association of Directors of Anatomic and Surgical Pathology (ADASP) consensus statement recommends. We have identified important areas of disagreement between pathologists and clinicians regarding what entities should be considered critical and who should be responsible for correlating histologic findings with the larger clinical context. Identifying these discordant points of view and fostering interdepartmental agreement on the best practices in the communication of critical diagnoses is an important patient care and safety issue. Chapman and Otis have also suggested the importance of increased access to accurate patient information and the clinical history, including the level of clinical suspicion of malignancy, and of forming a periodic review and quality assurance process. Here we explore methods of increasing the ability of pathologists and cytopathologists to identify unexpected diagnoses, including optimization of their workstations for better access to the electronic medical record, and we examine the progress of quality assurance methods in surgical pathology and cytopathology since the ADASP consensus statement in 2012.

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