Non-cirrhotic Portal Hypertension as the Initial Presentation of Limited Cutaneous Scleroderma: A Case Report.
Asif Ali HitawalaChristopher RedmondEdward W CowenDavid E KleinerSarfaraz HasniTheo HellerPublished in: Journal of investigative medicine high impact case reports (2023)
Systemic sclerosis (SSc) is an autoimmune disease characterized by progressive skin fibrosis. It has 2 main clinical subtypes-diffuse cutaneous scleroderma and limited cutaneous scleroderma. Non-cirrhotic portal hypertension (NCPH) is defined as presence of elevated portal vein pressures without cirrhosis. It is often a manifestation of an underlying systemic disease. On histopathology, NCPH may be found to be secondary to multiple abnormalities such as nodular regenerative hyperplasia (NRH) and obliterative portal venopathy. There have been reports of NCPH in patients with both subtypes of SSc secondary to NRH. However, simultaneous presence of obliterative portal venopathy has not been reported. We present a case of NCPH due to NRH and obliterative portal venopathy as a presenting sign of limited cutaneous scleroderma. The patient was initially found to have pancytopenia and splenomegaly and was erroneously labeled as cirrhosis. She underwent workup to rule out leukemia, which was negative. She was referred to our clinic and diagnosed with NCPH. Due to pancytopenia, she could not be started on immunosuppressive therapy for her SSc. Our case describes the presence of these unique pathological findings in the liver and highlights the importance of an aggressive search for an underlying condition in all patients diagnosed with NCPH.
Keyphrases
- systemic sclerosis
- interstitial lung disease
- blood pressure
- case report
- stem cells
- end stage renal disease
- multiple sclerosis
- ejection fraction
- newly diagnosed
- rheumatoid arthritis
- prognostic factors
- chronic kidney disease
- primary care
- emergency department
- cell therapy
- peritoneal dialysis
- computed tomography
- bone marrow
- arterial hypertension
- adverse drug
- pet ct