Professor of Pathology
Mayo Clinic College of Medicine

Rochester, Minnesota,
Consultant in the Department of Laboratory Medicine and Pathology
Mayo Clinic
Scottsdale, Arizona

Question: Our local pathologists are really excellent, but when one of my patients has suspected ILD and requires a surgical lung biopsy, they tend to send these out for expert review. Why is this the case?

Answer: The pathology of diffuse parenchymal lung disease is nearly always a manifestation of inflammatory/immune mechanisms. Diagnosing these conditions requires considerable experience. Several factors contribute to the diagnostic difficulty. First, the "images" of inflammatory diseases seen through the microscope are not specific for a single diagnostic entity and there is considerable overlap between inflammatory injuries.

Question: I have a patient with exposure to high levels (50+ ppm) of bromine vapor from a whirlpool. Patient experienced dermal burns (healed) and ongoing seizure, hallucination, memory problems, tremors, loss of taste, lumps under tongue and fissures on tongue, difficulty swallowing, shortness of breath, headaches, blood/oxygen level of 91, and rapid heart rate of up to 118 bpm at sitting. Patient cannot walk more than 100 ft. Is bromine exposure associated with an increased rate of IPF?

Answer: Acute bromine inhalation can result in acute toxic lung injury with chemical airway damage. It is conceivable that pulmonary injury in this setting could result in scar formation and compromised lung function. No data link toxic exposure to bromine and the idiopathic form of pulmonary fibrosis referred to as "IPF." Lung damage from any toxic exposure can result in permanent damage, including pulmonary fibrosis. This would not be "idiopathic" pulmonary fibrosis, but fibrosis related to acute bromine inhalation.

See abstract below:
Inagaki N, et al. Case with bromine exposure leading to respiratory insufficiency. Chudoku Kenyu. 2005 Apr;18(2):141-147.

Question: Is Agent Orange exposure a risk factor for IPF?

Answer: There are no available data directly linking exposure to Agent Orange with IPF. The etiology for IPF remains unknown. Epidemiologic studies suggest increased risk associated with metal and wood dust exposure, but even this is not evidence of causation.

Question: I have a 58 y/o male patient who has Sjögren's syndrome, factor V Leiden, IPF from a biopsy in 2005, and positive LAC. How commonly is IPF associated with autoimmune diseases (and is there a pathological connection)? This patient has a history of PE and clotting. Is lung transplant a viable option?

Answer: There is a definite pathological connection. Pulmonary fibrosis can occur in patients with Sjögren's syndrome and also in the context of many other "named" connective tissue diseases, with incidence varying according to diagnosis (eg, up to 40% of patients with scleroderma will develop interstitial lung disease). In this situation, a patient's pulmonary fibrosis would not be "idiopathic," however.

Lung transplantation may be an option, and will likely depend on the severity of any co-morbidities, the patient's performance status, the extent of lung disease radiologically, and the degree of pulmonary function deficit. The patient's Lupus anticoagulant status may play a role in this decision, as well.

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."