Extrapulmonary sarcoidosis with a focus on cardiac, nervous system, and ocular involvement

Abstract

Sarcoidosis is a poorly understood granulomatous disease that involves the lungs and/or intrathoracic lymph nodes in more than 90% of cases. Although pulmonary sarcoidosis is the leading cause of mortality in this disease, this review focuses on three sites of extrapulmonary involvement (heart, nervous system, and eyes), since involvement of any of these sites can be catastrophic, leading to death, debilitation, or blindness. Patients with cardiac, ocular and neurosarcoidosis necessitate a multidisciplinary approach with careful and long-term follow-up. Prompt diagnosis with imaging and/or biopsy and treatment is required to avoid irreversible damage. Corticosteroids are the mainstay of therapy and are often associated with rapid and durable remissions. Immunosuppressive or biologic agents are reserved for patients failing or experiencing side effects from steroids. Managing sarcoidosis requires vigilance, judgement, and awareness of the vagaries of this fascinating disease.

RETRACTED: Association between sarcoidosis and cardiovascular Outcomes: A systematic review and Meta-analysis

“…The characteristic features of sarcoidosis include bilateral hilar lymphadenopathy and reticular opacities in the lungs [3] , [4] . Sarcoidosis may also affect extrapulmonary organs like the heart leading to cardiac sarcoidosis (CS) [5] . While the causes of CS are currently unknown, the disease may be caused by over-reaction of the immune system post-exposure to infectious agents such as viruses or bacteria, allergens of chemicals [6] .…”

Study of CT-Guided Localization in Pulmonary Nodule Resection

“…If they cannot get effective treatment, the cure rate, survival rate, and pulmonary nodule deterioration rate of patients after operation will not be greatly improved, which will cause serious damage to the lungs and body of patients [ 8 ]. In the clinical medical research of pulmonary nodules, John et al believe that the traditional thoracotomy pneumonectomy has a serious loss of curative effect on patients with small cell lung cancer and has a great impact on the cure and recovery of patients after pneumonectomy [ 9 ]; Sahli et al pointed out in the study on the signs of other pulmonary sarcoidosis that due to the large chest opening, thoracotomy pneumonectomy not only increases the speed of wound healing but also takes a long time, which is extremely unfavorable to doctors and patients [ 10 ]; Francisco et al pointed out that multiple organs are involved in pulmonary sarcoidosis. Patients with pulmonary nodules undergoing routine surgery are very easy to be infected after operation, which not only causes serious loss of the whole lung but also related to injury to other parts of the body [ 11 ].…”

Epidemiology, Pathogenesis, and Diagnosis of Cardiac Sarcoidosis

“…Formation of discrete noncaseating granulomas consisting of epithelioid histiocytes and multinucleated giant cells surrounded by lymphocytes, plasma cells, and fibroblasts is the histological hallmark of sarcoidosis. 19 The multinucleated giant cells are initially foreign body type (with haphazardly arranged nuclei) and later become Langhans type (with peripherally arranged nuclei). They may contain cytoplasmic inclusions, particularly Schaumann bodies or asteroid bodies.…”

“…In CS, myocardium is usually involved with endocardium or epicardium as an extension of the disease. 19 , 20 , 25 Granulomas are frequently found in the basal interventricular septum, left ventricular (LV) free wall, right ventricle, and atria. 26 , 27 , 28 , 29 With disease progression, the granulomatous inflammation elicits a repair response with scarring, different from the scarring seen in myocardial infarction, which is patchy rather than maximal in the subendocardial region or transmural.…”