Abstract
Scleroderma (SSc) is an autoimmune disease that can result in end-stage interstitial lung disease (ILD). Rarely, SSc has been associated with primary sclerosing cholangitis (PSC). Lung transplant may be considered for the advanced treatment of SSc-related ILD. Herein, we describe the case of a lung transplant recipient with SSc who also presented a challenging diagnosis of PSC.
The patient is a 47-year-old female bilateral lung transplant recipient with history of SSc-related ILD and pulmonary hypertension. Maintenance immunosuppression consisted of tacrolimus, prednisone, and mycophenolate mofetil, and itraconazole was administered for anti-fungal prophylaxis. Early post-transplant, the patient experienced a tonic-clonic seizure related to tacrolimus toxicity. Decision was made to substitute cyclosporine, and levetiracetam was initiated for seizure prophylaxis. We then observed subtle elevations in her liver function tests. All medications were discontinued, without change in liver function, prompting MRI of the abdomen (Figure 1) which revealed multifocal areas of beaded appearance of the central intrahepatic ducts, suggesting PSC. Liver biopsy revealed focal portal tract fibrosis with bile duct lymphocytic exocytosis and vacuolar change, bile ductular proliferation, focal central venous minimal endothelialitis, and focal lobular necrosis with scattered lobular microgranulomata, with a differential of graft versus host disease. Ursodiol was started, and the patient underwent endoscopic retrograde cholangiopancreatography, which confirmed PSC. With no other interventions, liver function normalized.
Extrapulmonary sclerosis is a concern in lung transplant recipients with SSc. Esophageal involvement tends to garner the most attention from clinicians, yet this case raises awareness for PSC as a rare condition coincident with SSc. Medication use may be misleading when diagnosing the cause of elevated liver function.