Possible role of anti-SSA/Ro antibodies in the pathogenesis of pulmonary hypertension

Abstract

IntroductionThere are many different causes of pulmonary hypertension and the pathogenesis of the disease is still being elucidated. Although they are not the most common, autoimmunity and inflammation have been identified as possible causes. No one autoantibody has been identified as the definite cause of pulmonary hypertension. We present a rare association of anti-SSA/Ro antibodies and isolated pulmonary hypertension.Case presentationA 53 year old African American female presented with abdominal pain, nausea, weight loss, dyspnea and fatigue. Upon further exam she was found to have high titers of antinuclear antibodies and anti-SSA/Ro antibodies. This antibody profile would typically be suggestive of Sjögren's Syndrome, which is characterized by dry eyes and poor salivary gland function. However, since this patient did not have any symptoms consistent with the disease a diagnosis of Sjögren's Syndrome could not be made. A combination of laboratory, imaging and diagnostic studies were done that revealed a final diagnosis of pulmonary hypertension.ConclusionIt is known that pulmonary hypertension has association with autoimmune diseases, however no clear markers yet exist. Anti-SSA/Ro antibodies have been rarely described in cases of pulmonary disease, and less so in pulmonary hypertension. This case describes a unique association between isolated pulmonary hypertension and anti-SSA/Ro antibody, thereby illustrating the need to investigate this autoantibody and others in the pathogenesis of autoimmune pulmonary hypertension.

A Case of Sjögren’s Syndrome-Related Pulmonary Arterial Hypertension Treated with Iloprost and Bosentan Combination Therapy

“…Surprisingly, our patient did not have symptoms of SS, although he had ANA and anti-SSA antibody positivity. In the literature, there is only 1 case similar to ours reporting SRP without clinical manifestations despite serological positivity (4). The pathogenesis of PAH in SS is not clear.…”

The Spectrum of Extraglandular Manifestations in Primary Sjögren’s Syndrome

“…For long-QT syndrome diagnosis, ECG should be performed, while echocardiography with Tissue Doppler imaging and speckle-tracking technique can detect subclinical myocardial alterations [ 275 ]. When PH is suspected, right heart catheterization is recommended, and the diagnosis is defined as a pulmonary artery pressure above 25 mmHg [ 276 ]. Cardiac CT is recommended when pericardial involvement is suspected [ 255 ], while cardiac magnetic resonance (CMR) imaging [ 277 ] with tissue characterization sequences and positron emission tomography (PET) [ 278 ] provides additional insight regarding the presence of non-ischemic inflammatory myocardial involvement [ 279 ].…”

Pathology and Pathobiology of Pulmonary Hypertension

“…In addition, there is strong evidence that PAH involves alterations in multiple aspects of immunity that may contribute to the initiation and/or progression of pulmonary endothelial dysfunction and vascular remodeling. [172][173][174][175][176][177][178][179][180][181][182] Various stimuli such as altered hemodynamic conditions, dysfunctions in the BMPRII signaling system, chronic exposure to hypoxia, dysfunctional, or distressed mitochondria can modulate vascular inflammation. However, the sequence of events from inflammation initiation to pulmonary vascular disease development remains unknown in PAH.…”