Title: Revealing concealed Multisystemic Sarcoidosis presenting as Pacer induced Cardiomyopathy

Authors: Sabrina Afrin, MD, Rasik Neupane, MD, Ryan Toolson, DO, John Scrocco, MD

Email: safrin@mercy.com

Introduction: Sarcoidosis is an inflammatory, multisystemic disease affecting virtually any organ predominantly lungs, skin, lymphatics or eyes and is characterized by noncaseating granulomas [1]. Despite the efforts of several generations of research, understating of the mechanism and spectrum remains limited.[2] We explore a case of multisystemic sarcoidoisis.

Case report: A 59-year-old male with a history of hypertension, hyperlipidemia, and suspected obstructive sleep apnea presented with recurrent syncope and dyspnea. Initial findings included complete AV block, multilobular pneumonia, and mediastinal lymphadenopathy, leading to pacemaker implantation and antibiotic treatment. Despite normal initial LV function, the patient returned six months later with worsened respiratory symptoms. Persistent mediastinal and hilar adenopathy was noted, alongside deteriorated LV function 10-15%, prompting an upgrade to a dual chamber CRT-D for suspected pacemaker-induced cardiomyopathy. Cardiac MRI and further investigations confirmed cardiac sarcoidosis without significant coronary stenosis. Laboratory tests revealed low Vitamin D, mildly elevated calcium, and increased IL-2 levels. The patient was started on high-dose steroids and guideline-directed medical therapy, with a PET scan and endobronchial ultrasound biopsy confirming cardiac sarcoidosis and multisystem involvement. The treatment regimen included optimized steroids and comprehensive management for sarcoidosis, leading to his discharge on a tailored medication plan.

Discussion: Sarcoidosis, a complex multisystem disease, primarily targets lungs and lymph nodes but can affect any organ, leading to unpredictable symptoms and outcomes. Diagnosis relies on symptom compatibility, tissue granuloma detection, and excluding similar diseases, with chest imaging and biopsies playing key roles. Cardiac sarcoidosis, a significant mortality factor, necessitates advanced imaging like MRI or FDG-PET-CT for high-risk patients. Although biomarkers can suggest active inflammation, they are not conclusive. Management typically involves glucocorticoids, tailored to prevent organ failure and mitigate quality of life deterioration, highlighting the nuanced approach required for effective treatment.

Conclusion: Sarcoidosis presents an unpredictable natural history course, so understanding of pathophysiology and atypical or extrathoracic symptoms with multidisciplinary team involvement can lead to timely management and complications reduction.