Sudden death from ischaemic heart disease in a female patient with Sjögren syndrome

Abstract

Primary Sjögren syndrome (SjS) has a comparatively good prognosis except when it is complicated by non-Hodgkin's lymphoma. We performed an autopsy on a young female patient with primary SjS who had died suddenly during a meal, and we discuss the relationship between primary SjS and the cause of death. Eosinophilic change of the cardiomyocytes and severe arteriolosclerosis were observed within the myocardium. In the conduction system, lymphocyte infiltration was detected in the bundle of His, in addition to arteriosclerosis in the sinoatrial node and atrioventricular node arteries. The cause of death was diagnosed as ischaemic heart disease induced by arteriolosclerosis: its development can probably be attributed mostly to primary SjS. It should thus be kept in mind that primary SjS can occasionally result in the development of cardiovascular complications, such as ischaemic heart disease, as well as systemic lupus erythematosus or rheumatoid arthritis.

Cardiac arrhythmias as the initial manifestation of adult primary Sjögren's syndrome: a case report and literature review

“…However, the pathological basis of cardiac arrhythmias in pSS is far from clear. It is described an autopsy case following the sudden death of a young female patient with pSS, and the autopsy showed that the stenosis of the lumen in both sinoatrial (SA) node and AV node arteries and lymphocyte infiltration in the bundle of His, which may have led to fatal arrhythmias …”

“…It is described an autopsy case following the sudden death of a young female patient with pSS, and the autopsy showed that the stenosis of the lumen in both sinoatrial (SA) node and AV node arteries and lymphocyte infiltration in the bundle of His, which may have led to fatal arrhythmias. 12 The role of anti-SSA/SSB autoantibodies in the pathogenesis of heart block needs to be discussed. Maternal sera containing antibodies against SSA and SSB ribonucleoproteins (positive IgG) inhibited L-type Ca current in isolated cardiac myocytes and induced sinus bradycardia in a murine model of congenital heart block (CHB), and further study showed that maternal antibodies interact directly with the pore-forming alpha (1)-subunit of Ca channels.…”

Myocardial ischaemia reperfusion injury and cardioprotection in the presence of sensory neuropathy: Therapeutic options

“…Signs of cardiovascular autonomic nervous system dysfunction was detected in majority of patients affecting heart rate, BP variability, spontaneous baroreflex sensitivity and cardiovascular reflexes (Kovacs et al, 2004). Primary Sjögren syndrome may cause severe arteriosclerosis, development of ischaemic heart disease and occurrence of sudden cardiac death (Inoue et al, 2008). Like any other complex human disease, Sjögren's syndrome has no single animal model which could replicate all aspects of the disease.…”

Ventricular ectopy after exercise and occult ischaemia in a patient with multiple risk factors for ischaemic heart disease and defective anginal warning system

“…He had a history of Sjögren's syndrome, diabetes, dyslipidemia, hypertension, chronic obstructive pulmonary disease and was under methotrexate treatment for rheumatoid arthritis. Such diseases imply a severe dysfunctional burden, tend to develop accelerated atherosclerosis, endothelial dysfunction and ischaemic heart disease (IHD) 1 2. A functional assessment was thus provided with an exercise/rest technetium-99 m tetrofosmin-gated single-photon emission cardiac tomography (G-SPECT).…”