Review
Sarcoidosis: Challenging Diagnostic Aspects of an Old Disease

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Abstract

Over the past few years, there have been substantial advances in our understanding of sarcoidosis immunopathogenesis. Conversely, the etiology of the disease remains obscure for a number of reasons, including heterogeneity of clinical manifestations, often overlapping with other disorders, and insensitive and nonspecific diagnostic tests. While no cause has been definitely confirmed, there is increasing evidence that one or more infectious agents may cause the disease, although the organism may no longer be viable. Here we present 2 cases, in which sarcoidosis preceded tuberculosis and non-Hodgkin lymphoma. Development of new lesions in a patient with chronic/remitting sarcoidosis should be looked at with suspicion and promptly investigated in order to rule out an alternative/concomitant diagnosis. In such cases, tissue confirmation from the most accessible site, and bone marrow biopsy—if lymphoma is in the differential diagnosis—should be performed. In conclusion, we strongly advise that physicians be ready to reconsider the diagnosis of sarcoidosis in the presence of atypical manifestations or persistent/progressive disease despite conventional therapy.

Section snippets

Clinical Case 1

A 58-year-old man, a lifelong nonsmoker, had been previously diagnosed with sarcoidosis (based on transbronchial biopsies showing epithelioid non-necrotizing granulomas and compatible clinicoradiologic features) and successfully treated with oral steroids for 6 months (Figure 1). At that time, tuberculin skin test was negative. Nine years later he presented with 3 months history of night fever (up to 39°C), abdominal pain (left upper quadrant), fatigue, and weight loss. The patient was referred

Clinical Case 2

A 76-year-old woman, a lifelong nonsmoker, presented to our chest clinic with persistent high fever (up to 40°C). She had been diagnosed 12 months earlier with a cutaneous diffuse follicular lymphoma, which did not require any systemic treatment. Chest radiograph and computed tomography (CT) scan showed multiple enlarged mediastinal and hilar lymph nodes (Figure 3A). She underwent bronchoscopy with BAL, which revealed mild lymphocytosis (18%) with a normal CD4/CD8 ratio, and multiple

Concluding Remarks

The diagnosis of sarcoidosis requires the presence of a compatible clinical/radiographic picture, histologic evidence of noncaseating granulomas, and exclusion of known causes of granulomatous inflammation. Sarcoid granulomas can occur with varying rates in any organ system, but are most commonly found in the lungs and lymph nodes. Patients may be entirely asymptomatic and the condition discovered by the presence of characteristic findings on routine health screening chest radiographs (30%-60%

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    Conflict of Interest: None.

    Authorship: All authors had access to the data and a role in writing the manuscript.

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