Sarcoidosis: impact of other illnesses on the presentation and management of multi-organ disease

Abstract

Sarcoidosis remains a fascinating illness that almost always affects the respiratory tract but often involves many other organs as well. Although many patients seem to have only an intrathoracic illness, with perhaps one other site or organ involved, others can experience a severe multi-organ disease. The inciting stimulus, even if unknown, can elicit an immunologic host response-the non-caseating granuloma-in almost every organ. It is intriguing that this stimulus can be so widespread throughout the body, while the biology of the disease can be so variable. Many series of patients with sarcoidosis have reported the multiple organs involved and the clinical presentation. Our series of 67 patients (40 female, 27 male, mean age 38.7 years +/- 13.2 (SD) at time of diagnosis) generally mirrors the clinical pattern found in five comparison series that span the past 60 years. However, more emphasis is given in this series to associated medical conditions that can complicate the presentation of sarcoidosis, as well as to co-morbid illnesses that must be managed in addition to the patient's sarcoidosis. Although most patients had intrathoracic sarcoidosis diagnosed at initial evaluation (40%), many had other organs or bodily sites involved in addition (or subsequently) as the illness evolved. Confounding the initial patient evaluation were two factors: (1) the presence of an occupational respiratory exposure(s) (n = 25 or 37% of patients); (2) a previously diagnosed malignancy (n = 6 or 9%) that heightened the possibility of a primary malignancy presenting in the chest, or the reactivation of a prior malignancy (breast, thyroid, and lymphoma) that could metastasize to the lung. Symptoms present when a patient's diagnosis was established usually differentiated respiratory and/or abdominal organ involvement. Although respiratory symptoms could be absent (n = 18 or 27%) for many patients with incidental thoracic findings, most had typical ones, including exertional dyspnea. For patients with an abdominal presenting illness (n = 11 or 16%), nonspecific digestive and abdominal symptoms were experienced as well as arthralgias. Almost every patient had at least one important other illness that factored significantly into the management of their sarcoidosis. Older patients had more illnesses, such as cardiovascular illness, diabetes mellitus, neurologic problems, and functional gastrointestinal symptoms. Depression affected all ages and was probably underrecognized; more emphasis on this illness is needed. Obesity was associated with disordered sleep syndromes, but not invariably so, as half the subjects had a good body habitus. Thus, many of the other illnesses experienced by sarcoidosis patients are common problems that middle-aged people develop. However, digestive and gastroenterological symptoms seemed disproportionately frequent in this series. This is a component of multi-organ sarcoidosis that has not received extensive coverage in the literature. Approximately one-third of sarcoidosis patients had one of two very common problems-gastroesophageal reflux or irritable bowel syndrome. But these are common problems, and it is thus necessary to separate these symptoms from those associated with abdominal visceral involvement of sarcoidosis. Although liver and/or splenic involvement with sarcoidosis do not cause organ dysfunction or insufficiency, they can contribute to abdominal symptoms. Finally, it remains of interest whether inflammatory bowel disease-Crohn's disease in particular-is another organ manifestation of sarcoidosis, or is it unrelated?