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Telomeres are repetitive nucleotide sequences that prevent chromosomal shortening in cell replication. Short telomeres have been implicated in the pathogenesis of interstitial lung disease. Patients with short telomere related pulmonary fibrosis can have computed tomography (CT) findings inconsistent with pro‐typical usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF) pattern. They can have rapid progression and overall worse prognosis. Antifibrotic drugs, like pirfenidone, can be used to slow the progression of disease, but there is conflicting data in patients with Telomerase reverse transcriptase/Telomerase RNA component (TERT/TERC) mutations, hence genetic testing plays an important role in determining the therapeutic options. These patients should be referred for lung transplantation early. We present a case of rapidly progressive pulmonary fibrosis associated with short telomere., Short telomeres have been associated with familial pulmonary fibrosis. Genetic testing is essential to determine therapeutic options and perform appropriate screening

An 84-year-old woman with a medical history of hypertension, hypothyroidism, and transient ischemic attacks presented with right-sided upper and lower extremity weakness for 1 day. She was a lifetime nonsmoker with no known heart disease or chronic lung disease. She had no occupational exposure or pertinent family history. She denied any fevers, chills, rashes, or shortness of breath. There was no history of recent travel.