A case study diagnosing sarcoidosis upon autopsy in a patient with acute respiratory distress syndrome (ARDS) of unknown cause highlights the importance of physicians taking into account this uncommon disease association for quicker diagnosis and treatment.
The study, “Sarcoidosis Presenting as Acute Respiratory Distress Syndrome,” was published in the journal Case Reports in Medicine.
Sarcoidosis is an inflammatory disease of unknown cause that can affect several organs in the body, especially the lungs, a condition known as pulmonary sarcoidosis. Patients with pulmonary sarcoidosis can experience shortness of breath, fatigue, chest pain, and abnormal breathing.
Acute pulmonary conditions such as ARDS are uncommon, and when they occur, they often affect patients with advanced sarcoidosis. ARDS is characterized by rapid respiratory failure caused by infections by microorganisms (bacteria, fungi, or viruses) in the blood or pneumonia (infection of the lungs), among others. Survival expectancy in patients with ARDS is generally low.
Only a few publications have reported cases of ARDS-associated sarcoidosis.
To highlight the rare but potential presentation of sarcoidosis in ARDS patients, the researchers here reported the case of a patient with ARDS of unknown cause who was diagnosed with sarcoidosis after her autopsy.
The patient was a 41-year-old African-American woman who went to the emergency roomafter experiencing cough, shortness of breath, fevers, chills, night sweats, and fatigue. She showed a sudden development of a cough and fever that did not reduce in intensity even after one week of antibiotic treatment for pneumonia and was admitted to the hospital.
The medical team diagnosed her with ARDS, based on the Berlin definitions — internationally agreed-upon criteria for the diagnosis of ARDS.
However, initial assessment of the patient showed no signs of infections by microorganisms or parasites. Three days after being hospitalized, her symptoms worsened, and the team further assessed the presence of microorganisms, this time also testing for different types of viruses. Collectively, the results showed no signs of infections.
The patient’s condition progressively worsened, resulting in increased hypoxia (low oxygen in the tissues) and problems breathing, ultimately requiring mechanical breathing and inhaled epoprostenol — a vasodilator (blood-vessel opening therapy). With time, the patient developed liver and kidney complications, which required hemodynamic (blood flow) support.
The patient died as a result of worsening ARDS and right heart failure.
A subsequent autopsy showed that the patient had granulomas in her lymph nodes, lungs, spleen, and liver. After excluding other granuloma-associated diseases, the final diagnosis was confirmed as sarcoidosis.
“This is the only reported case of ARDS from an acute febrile presentation of sarcoidosis which was diagnosed posthumously,” the researchers wrote. “Sarcoidosis is not a usual cause of ARDS and is seldom cited as the underlying pathology in these patients; as our case and other cases before illustrated, it is essential to consider this in the differential for unexplained ARDS.”
They concluded that “acute respiratory failure due to ARDS is an underrecognized presentation of sarcoidosis,” and suggest that it “can be potentially fatal if not treated promptly. Physicians need to be vigilant in considering sarcoidosis as a cause of ARDS after excluding common etiologies.”