Sarcoidosis is an inflammatory disease of unknown cause, in which granulomas (clumps of inflammatory cells) form in one or more organs in the body. Early symptoms of sarcoidosis can include a skin rash, swollen or painful joints, fever, or enlarged lymph nodes. Symptoms of advanced sarcoidosis can include fatigue, irregular heartbeat, visual problems, weakness or numbness in the limbs or parts of the face, and unexplained weight loss. Disease characteristics and severity of sarcoidosis can vary greatly from patient to patient.

While it can affect any organ in the body, the lungs are affected in 90% of patients with sarcoidosis. While spontaneous remission occurs in a majority of sarcoidosis cases, chronic inflammation in the lungs can lead to pulmonary fibrosis. Once scar tissue is present in the lungs, it cannot be reversed.

Supplemental oxygen, pulmonary rehabilitation, and management of symptoms are important treatment options for many types of pulmonary fibrosis, depending on how severe the disease is. Smoking cessation and routine vaccinations are important parts of living with pulmonary fibrosis. Treatments specific to sarcoidosis-associated pulmonary fibrosis can include medications like corticosteroids and immunosuppressants.

Lymphangioleiomyomatosis (LAM) is a rare disease that affects multiple organ systems in the body and is found almost exclusively in women. In LAM, smooth muscle cells grow at an abnormal rate, particularly in the lungs, kidneys, and lymphatic system. Those affected by LAM are grouped into two categories: sporadic LAM, which occurs for unknown reasons; and LAM caused by tuberous sclerosis complex (TSC), a rare disease. Uncontrolled growth of smooth muscle cells in lung tissue due to LAM can block airways and release fluid into the lungs that can result in cystic lung damage.

While there currently is no cure for lung disease associated with LAM, there are medications (sirolimus and everolimus) that may improve lung function in some patients living with LAM. Supplemental oxygen, pulmonary rehabilitation, and management of symptoms are important treatment options for many types of pulmonary fibrosis, depending on how severe the disease is. Smoking cessation and routine vaccinations are important parts of living with pulmonary fibrosis. Lung transplantation may be an option for some patients with LAM-associated pulmonary fibrosis.

“Interstitial lung disease” refers to a large group of respiratory diseases that cause inflammation and / or scarring in the lung tissue, or “interstitium”. When an ILD includes scar tissue in the lung, we call it pulmonary fibrosis. Some patients with interstitial lung disease do not fall into a specific category of pulmonary fibrosis, even with multidisciplinary discussion among expert pulmonologists, radiologists, and pathologists. A patient may be diagnosed with “unclassifiable interstitial lung disease” if they do not meet all the criteria to be confidently diagnosed with a specific type of interstitial lung disease or pulmonary fibrosis. This occurs when there are nonspecific or conflicting findings on their CT scans, lung biopsies, or in the clinical course of the disease. Another case when a diagnosis of unclassifiable ILD may occur is when the patient is unable to go through standard diagnostic tests.

Supplemental oxygen, pulmonary rehabilitation, and management of symptoms are important treatment options for many types of pulmonary fibrosis, depending on how severe the disease is. Lung transplant may be an option for some patients with unclassifiable ILD. Smoking cessation and routine vaccinations are important parts of living with pulmonary fibrosis.