Saturday, 3 September 2011

Sarcoidosis - Unusual manifestations that challenge clinicians to diagnose and establish treatment for these problems

Correspondence: D.R. Moller, Division of Pulmonary and Critical Care Medicine, Dept of Medicine, The Johns Hopkins University, 5501 Hopkins Bayview Circle,Rm. 4B63, Baltimore, MD 21224USA. E-mail:


Although most sarcoidosis patients develop manifestations within a clinical framework typical for sarcoidosis, a small number of patients develop unusual manifestations that challenge clinicians to diagnose and establish treatment for these problems. These rare manifestations are usually the result of either an uncommon pattern of systemic involvement or granulomatous inflammation developing in an unusual location for sarcoidosis. Despite being rarely seen, these clinical manifestations reflect the same underlying pathophysiological mechanisms and clinical behaviour common to more easily recognisable systemic sarcoidosis. Similarly, the known T-helper cell type 1 (Th1) polarisation in sarcoidosis provides the clinician with a reminder that Th1-promoting therapeutics, such as interferon-α, common variable immunodeficiency or immune reconstitution in HIV patients, are associated with development of sarcoidosis. Sarcoidosis may also be associated with autoimmune disease or cancer, although this is rare.
An approach to sarcoidosis patients with possible rare manifestations must be individualised according to whether a diagnosis of sarcoidosis has already been established or not. As an initial step, an internet search may substantiate previous experience with similar manifestations. The clinician will often need to: 1) recommend additional diagnostic testing or biopsy, unless there is already biopsy-proven systemic sarcoidosis; and 2) check the rare manifestation is consistent with sarcoidosis and that the chance of an alternative diagnosis is low. In this latter situation, the clinician may recommend a trial of corticosteroid treatment to support an association with sarcoidosis. In patients without a confirmed diagnosis of sarcoidosis, a directed evaluation with biopsy is usually indicated.
In all situations, the clinician must remain vigilant that unusual manifestations of sarcoidosis may, in fact, represent an alternative condition. Although the diagnostic approach to rare manifestations must be individualised, the patient and clinician are usually rewarded by establishing a link to sarcoidosis so that treatment specific to sarcoidosis can be tailored for maximal benefit.
© 2011 European Respiratory Society

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