Comorbidities of sarcoidosis

Abstract

Sarcoidosis is a heterogeneous disease, which can affect virtually every body organ, even though lungs and intra thoracic lymph nodes are almost universally affected. The presence of noncaseating granulomas is the histopathological hallmark of the disease, and clinical picture depends on the organs affected. Data about interaction between sarcoidosis and comorbidities, such as cardiovascular and pulmonary diseases, autoimmune disorders, malignancy and drug-related adverse events are limited. Several lung conditions can be associated with sarcoidosis, such as pulmonary hypertension and fibrosis, making it difficult sometimes the differentiation between complications and distinctive pathologies. Their coexistence may complicate the diagnosis of sarcoidosis and contribute to the highly variable and unpredictable natural history, particularly if several diseases are recognised. A thorough assessment of specific disorders that can be associated with sarcoidosis should always be carried out, and future studies will need to evaluate sarcoidosis not only as a single disorder, but also in the light of possible concomitant conditions.Key messagesComorbidities in sarcoidosis are common, especially cardiovascular and pulmonary diseases.In the diagnostic workup, a distinction must be made between sarcoidosis-related complaints and complaints caused by other separate disorders. It can be very difficult to distinguish between complications of sarcoidosis and other concomitant conditions.The coexistence of multiple conditions may complicate the diagnosis of sarcoidosis, affect its natural course and response to treatment.

Keywords: Adverse drug reactions; comorbidity; heart disease; multidisciplinary management; pulmonary hypertension; sarcoid-like reaction; sarcoidosis; treatment.

Figure 1.

SAPH due to extrinsic vascular compression. CT scan shows compression of the right mediastinal pulmonary artery by enlarged lymph nodes. Note the increased diameter of main pulmonary artery as compared to aorta diameter.

Figure 2.

Screening and diagnosis of SAPH [8,11]. *Decision to proceed with right heart catheterisation should be discussed by a multi-disciplinary team with a sarcoidosis and a PH expert and take into account potential clinical consequences, and lung function. † Additional echocardiographic signs suggestive of PH [10]. ^¶In cases with a high index of clinical suspicion, right heart catheterisation should be considered on a case-by-case basis. SAPH: sarcoidosis-associated pulmonary hypertension, 6MWT: six minutes’ walk test, FVC: forced vital capacity, DLCO: diffusing capacity for carbon monoxide, PA: main pulmonary artery, BSA: body surface area, CTA: computed tomography angiography, TRV: peak tricuspid regurgitation velocity, RHF: right heart failure; LVD: left ventricular dysfunction, mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure, SAPH: sarcoidosis-associated pulmonary hypertension.

Figure 3.

UIP-like comorbidity in a patient with sarcoidosis. CT scan shows a UIP-like pattern, with reticulations, bronchiectasis, and honeycombing with a basal and peripheral predominance in a patient with confirmed sarcoidosis.