**2. Diagnosis**

The diagnosis is based on clinical and imaging features, histological confirmation, and exclusion of other diseases that can create similar histopathological and clinical findings.

Imaging is mandatory for diagnosis of sarcoidosis and includes as follows:


### **2.1 Chest X-rays and HRCT scan**

A correct staging of pulmonary sarcoidosis based on radiological stage of the disease is necessary to evaluate the sarcoidosis prognosis [1]. Particularly, there are five radiologic stages (forms) of intrathoracic sarcoidosis on chest radiography [2–4] as follows:


**33**

*Early Diagnosis of Sarcoidosis*

*DOI: http://dx.doi.org/10.5772/intechopen.90523*

• Stage IV: pulmonary fibrosis.

older than 50 years old of age;

• pulmonary ground glass opacity;

• consolidation or large nodular opacities;

zones and with subpleural involvement.

**2.2 Cardiopulmonary and laboratory tests**

distribution;

cysts;

and

accepted HRCT scoring system.

infiltrates with parenchymal lesion;

• Stage III: infiltrates alone, parenchymal disease only; and

Main findings of HRCT sarcoidosis are as follows:

• thickening of the peribronchovascular interstitium;

• Stage II: bilateral mediastinal lymphadenopathy (hilar lymphadenopathy) and

HRCT, however, is more accurate in identifying the different manifestations of pulmonary sarcoidosis as well its complications, but we are still searching for an

• hilar and/or mediastinal lymph node enlargement (sometimes with calcifications), classically bilateral hilar and right paratracheal nodal enlargement (Garland triad); left paratracheal and aortopulmonary nodes can be also enlarged. Atypical pattern of nodal enlargement can be observed in patients

• pulmonary interstitial nodules (micro or macronodules) in a perilymphatic

• coarse linear opacities, interlobular septal thickening, honeycombing, and

• architectural distortion, superior hilar retraction, or traction bronchiectasis;

• pulmonary fibrosis (Stage IV) with linear bands of fibrosis from hila to all directions with distortion of normal lung architecture; honeycombing is rare, only in patients with severe fibrosis, mainly in the middle and upper lung

The diagnosis of pulmonary sarcoidosis requires a compatible clinical picture supported by radiologic (X-rays chest and/or HRCT of lungs) and pathologic data (pulmonary functional tests and laboratory tests). But for a certain diagnosis, biopsy is required in most cases, and endobronchial biopsy via bronchoscopy is often performed; its results may be positive even in patients with normal chest radiographs. The central histologic finding is the presence of noncaseating granulo-

Pulmonary function tests are also routinely used in sarcoidosis for early diagnosis and follow-up. They include the evaluation of forced vital capacity (FVC), forced expiratory volume in one second (FEV1), the total lung capacity determination (TLC), vital capacity (VC), residual volume (RV) associated with body

Pleural disease is rare and may be observed when disease is extensive.

mas with special stains negative for fungi and mycobacteria [2].

*Sarcoidosis and Granulomatosis - Diagnosis and Management*

and scleral plaques.

production (rare) [2].

**2. Diagnosis**

clinical findings.

and biopsy yield [2]; and

**2.1 Chest X-rays and HRCT scan**

• Stage 0: normal chest radiographic findings;

therapy) [3].

[2–4] as follows:

patients, but quite uncommon in African-American and Japanese patients.

*Cutaneous involvement* may be present not only with erythema nodosum associated with Löfgren syndrome, but also with lupus pernio, violaceous rash on the cheeks or nose (quite common) and maculopapular plaques (quite uncommon) [2].

*Ocular involvement* is also possible, which may lead to blindness for untreated anterior or (most frequent) posterior granulomatous uveitis, conjunctival lesions

Other uncommon possible manifestations are [2] *nervous system involvement* with lymphocytic meningitis (rare), cranial nerve palsies, hypothalamic/pituitary dysfunction (rare) with diabetes insipidus and myelopathy; *heart failure* from cardiomyopathy (rare) or heart block and sudden death; *osseous involvement* with arthritic syndromes; *blood abnormalities*: anemia, leukopenia, thrombocytopenia and hemolytic anemia with or without splenomegaly (without splenomegaly may reflect bone marrow involvement); *gastrointestinal and genitourinary involvement* (rare): hepatomegaly, cholestasis, portal hypertension, Crohn's disease, pancreatic involvement, nephrocalcinosis, vulva itchiness and male infertility (rare); *exocrine and endocrine manifestations* with hyperprolactinemia, amenorrhea, galactorrhea, or nonpuerperal mastitis in women; hypercalciuria and hypercalcemia likely result from the increased 1,25-dihydroxy vitamin D

The diagnosis is based on clinical and imaging features, histological confirmation, and exclusion of other diseases that can create similar histopathological and

• routine chest computed tomography (CT), usually high-resolution CT (HRCT) scanning of the chest [2] (has a significantly superior detection rate to chest X-ray for mediastinal and pulmonary parenchymal changes and is also useful to identify active alveolitis or fibrosis), and the agreement between findings

• fluoro-deoxy glucose-positron emission tomography (FDG-PET) combined with CT-scan (is fundamental to determinte the extent of organ involvement and the disease activity and to evaluate the response to pharmacological

A correct staging of pulmonary sarcoidosis based on radiological stage of the disease is necessary to evaluate the sarcoidosis prognosis [1]. Particularly, there are five radiologic stages (forms) of intrathoracic sarcoidosis on chest radiography

• Stage I: bilateral mediastinal lymphadenopathy (hilar lymphadenopathy);

Imaging is mandatory for diagnosis of sarcoidosis and includes as follows:

• chest radiography (is fundamental for a first evaluation);

**32**


HRCT, however, is more accurate in identifying the different manifestations of pulmonary sarcoidosis as well its complications, but we are still searching for an accepted HRCT scoring system.

Main findings of HRCT sarcoidosis are as follows:


Pleural disease is rare and may be observed when disease is extensive. The diagnosis of pulmonary sarcoidosis requires a compatible clinical picture supported by radiologic (X-rays chest and/or HRCT of lungs) and pathologic data (pulmonary functional tests and laboratory tests). But for a certain diagnosis, biopsy is required in most cases, and endobronchial biopsy via bronchoscopy is often performed; its results may be positive even in patients with normal chest radiographs. The central histologic finding is the presence of noncaseating granulomas with special stains negative for fungi and mycobacteria [2].
