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Cardiac sarcoidosis (CS) is an important cause of mortality in patients with sarcoidosis. The aim of this retrospective cohort study was to characterize the prevalence, incidence, clinical features and outcomes of CS in the southern region of New Zealand. 45 patients were identified: 23 fulfilling international classification criteria, 9 fulfilling physician consensus criteria, and 13 classified as possible CS. As of June 2021, 26 patients were living and domiciled in the Canterbury district; estimated point prevalence was 4.43 cases per 100,000 people. The average annual incidence was 0.24 cases per 100,000 people between 2016 and 2020. We estimated a 5.14% frequency of CS in patients with sarcoidosis. Median age at presentation was 56 years (range 31–72). Common presentations included heart failure, heart block and life threatening ventricular and supraventricular arrhythmias. Electrocardiogram abnormalities were found in 93.3% and cardiac MRI was often relied upon by physicians for diagnosis. The 10-year survival was 94% (95% CI 78–99%). Our study provides further insight into the epidemiology of CS. In this retrospective cohort the frequency of CS amongst patients with sarcoidosis was estimated at 5%, whilst the estimated point prevalence of the disease was twice that of a contemporary report from the Northern hemisphere. The 10-year survival was similar to contemporaneous reports from other developed countries. • The population prevalence of cardiac sarcoidosis was 4.43 cases per 100,000 people. • Average annual incidence between 2016 and 2020 was 0.24 cases per 100,000 people. • An estimated 5% of people with sarcoidosis had clinical cardiac involvement. • The 10-year survival rate of 94% was similar to that of other developed countries. [ABSTRACT FROM AUTHOR]

Aims: We describe the varied clinical presentations, barriers in diagnosis and outcomes of anti-HMGCR myopathy in a large national cohort., Methods: Adults found positive for serum anti-HMGCR autoantibodies via line blot or enzyme-immunoassay followed by immunoprecipitation were included in the study., Results: Of 75 patients identified, the records of 72 (96 %) described weakness as the presenting symptom. The records of 65 gave a reliable description of proximal weakness. In 22/65 (33.8 %) the weakness was described as predominantly or solely lower limb weakness. Forty-five of 75 (60 %) presented with a subacute onset (duration of symptoms >4 weeks -≤6 months), whilst 22/75 (29.3 %) presented with a more indolent chronic onset (duration of symptoms >6 months). Eighteen of 75 (24 %) suffered falls and 2/75 (2.7 %) had "general decline". In three patients no weakness was described: two presented with myalgia and one with a skin rash characterized as Jessner lymphocytic skin rash. Median creatine kinase at presentation was 7337 U/L (range 1050-25,500). Muscle biopsy was performed in 38 (50.7 %). Associated malignancy was infrequent. Four patients recovered without immunosuppression. Five-year and 10-year survival was 92.7 % (95 % CI 80.6-97.4 %), and 82.5 % (95 % CI 61.2-92.8 %) respectively., Conclusion: Recurrent falls, a long prodrome and dominant lower limb proximal weakness were common in this anti-HMGCR myopathy cohort. These features overlap with frailty syndrome and sporadic inclusion body myositis emphasizing the importance of considering anti-HMGCR myopathy in that clinical context. A minority of patients recover after statin withdrawal alone., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)