Multiorgan Involvement: Is There a Relevant Pathogenetic Link?

Abstract

Multisystem autoimmune disorders have different presenting symptoms with organ involvement phased over numerous years. We have a 56-year-old homemaker who is a known case of Graves' disease-post-thyroidectomy performed 20 years ago-and developed a volume overload state with exertional dyspnea for a period of 1.5 years. She was evaluated elsewhere and diagnosed with pulmonary arterial hypertension (PAH), the cause of which had not been established at the time. She presented to us with progressive dyspnea and abdominal distension of 1-week duration. A further diagnosis of biopsy-proven autoimmune hepatitis, chronic parenchymal liver disease (CLD), and noninfiltrative restrictive cardiomyopathy (RCM) was made. At follow-up, the patient complained of thickening and dryness of skin with similar antinuclear antibody (ANA) profile results 6 months apart. In the background of immune-mediated organ manifestations and laboratory picture, systemic sclerosis was diagnosed. This case report highlights that one should have a high index of suspicion while dealing with subsequent autoimmune manifestations of isolated organs as they might be part of a multisystem autoimmune disorder.