Wallach's Interpretation of Diagnostic Tests: Pathways to Arriving at a Clinical Diagnosis (575 page)

   Clinical presentation of sarcoidosis is variable and depends on ethnicity, duration of illness, site and extent of organ involvement, and activity of the granulomatous process.

   Sarcoidosis typically presents with bilateral hilar lymphadenopathy, pulmonary infiltration, and skin and ocular lesions.

   Sarcoidosis can be clinically classified as

   
Asymptomatic sarcoidosis
: Incidentally detected on chest imaging. Thirty to 50% of patients found to be asymptomatic at the time of diagnosis.

   
Sarcoidosis with nonspecific constitutional symptoms
: Observed more frequently in African Americans and Asian Indians. The nonspecific symptoms include fever (39–40°C), weight loss (2–6 kg), fatigue, and malaise.

   
Sarcoidosis with symptoms related to specific organ involvement
: Acute sarcoidosis has sudden onset, more frequently seen in Caucasians and may part as Lofgren syndrome (bilateral hilar adenopathy, erythema nodosum, and ankle arthritis) and constitutional nonspecific symptoms. Organ-related symptoms, often related to pulmonary infiltration (cough and dyspnea).

   
Pulmonary sarcoidosis
: Asymptomatic (30–60%), but chest radiograph abnormalities are high (85–95%). Clinical course is very heterogenous, with 2/3 patients show spontaneous remissions and can be chronic and progressive about 10–30% of patients causing destruction of lung and permanent loss of lung function. Seventy-five percent of patients have bilateral lymphadenopathy.

   
Extrapulmonary:
Common but is almost always associated with lung involvement. May involve entire length of respiratory tract airways causing obstructive airway disease and broad spectrum of airway dysfunction. Common in African Americans than in Caucasians, and in addition, the eyes, bone marrow, extrapulmonary lymph nodes, and skin are more frequently involved.

   
Cutaneous disease:
Skin lesions can be divided into specific and nonspecific on the basis of the presence or absence of granulomatous inflammation on histopathology. Erythema nodosum, lupus pernio, and violaceous rash on the cheek or more are common.

   
Ocular disease:
Most common ocular manifestation is anterior uveitis, which can manifest with blurred vision, red, painful eyes, and photophobia. Conjunctiva can be affected in 6–40% of cases. Optic neuropathy is rare but can cause rapid, permanent loss of vision or color vision.

   
Hepatic disease:
Hepatic sarcoidosis is usually asymptomatic, but the common features are abdominal pain, pruritus, fever, weight loss, and jaundice. Biopsy-based studies showed presence of granulomas is 50–65% of patients and serology-based studies showed abdominal liver function tests in 35% of patients.

   
Cardiac disease
: Known to give rise to heart failure, arrhythmias, sudden cardiac death, and granulomatoses, inflammation in the heart is present in 25% of patients.