Question: I am treating a 32 y/o Hispanic man with pulmonary fibrosis and a pathology result consistent with UIP with lymphoid hyperplasia. His tests for connective tissue diseases have all been negative. He has worked in construction in the past. He has no insurance and no medical coverage. I placed him on prednisone 30 mg po qd and TMP-SMX mwf for pcp prophylaxis because this is an atypical case (young, lymphoid hyperplasia) on the off chance that he has an atypically steroid responsive process (though UIP usually isn't). Do you know of any data regarding treatment of cases like this?

Answer: The diagnosis of "UIP" in a 32 y/o is unlikely to correspond to idiopathic pulmonary fibrosis, as we understand that condition today. Clearly he must have advanced fibrosis on biopsy, and this has resulted in the pathological diagnosis of "UIP."

UIP-pattern lung fibrosis accompanied by lymphoid hyperplasia in a 32 y/o raises a differential diagnosis to include: 1) clinically occult systemic autoimmune disease manifesting as pulmonary fibrosis; 2) chronic medication toxicity (most often to very specific drugs such as nitrofurantoin); and 3) "familial" forms of pulmonary fibrosis.

The distribution on HRCT will be very important in establishing prognosis, especially in conjunction with his baseline pulmonary function (including data on desaturation with exercise). I would make sure that a broad panel of serologic studies has been performed, even if a rheumatic condition is not clinically apparent (ANA, RF, ANCA, SCL70, SSA, SSB, aldolase, Jo-1, sed rate, CRP).

Many pulmonologists who are experts in the care of patients with ILD would agree with your approach to therapy in this patient, in fact some would strongly advocate the use of a combined therapy approach with the addition of a cytotoxic agent such as azathioprine (see ATS/ERS consensus statement on IPF for dosage and administration), with or without N-acetylcysteine (NAC). It is doubtful that any therapy will reverse existing fibrosis in this patient, but by modifying or eliminating an immunologically mediated mechanism, the overall prognosis may be changed favorably.