Page 1023 - Clinical Immunology_ Principles and Practice ( PDFDrive )

986 Part seven Organ-Specific Inflammatory Disease

Extrapulmonary Sarcoidosis
Most patients have clinically important involvement of more
than one organ, either with or without pulmonary sarcoidosis
(see Table 73.1).
Sarcoidosis of the Upper Respiratory Tract
Sarcoidosis can cause nasal congestion, crusting, epistaxis,
anosmia, rhinorrhea, sinusitis, or nasal septal perforation; a
“saddle nose” deformity, palatal perforation, or bone erosion
may occur. Laryngeal sarcoidosis occurs in 1–5% of patients,
with hoarseness, dysphonia, dysphagia, dyspnea, or, rarely, stridor
and acute respiratory failure.
Ocular Sarcoidosis
Unilateral or bilateral anterior uveitis is the most common
manifestation of ocular sarcoidosis, often in association with
bilateral hilar adenopathy. Chronic uveitis occurs in up to 20%
patients with chronic sarcoidosis, more frequently in Black
populations. Other manifestations include posterior uveitis,
granulomatous conjunctivitis, severe chorioretinitis, or optic
neuritis that can cause blindness (Chapter 74).
Cutaneous Sarcoidosis
Erythema nodosum, which is a feature of Löfgren syndrome, may
recur. Chronic skin sarcoidosis usually manifests as nontender,
nonpruritic plaques and subcutaneous nodules around the hair-
line, eyelids, ears, nose, mouth, and the extensor surfaces of arms
and legs. Lupus pernio is a disfiguring form of facial cutaneous
sarcoidosis, with violaceous plaques and nodules covering the
FIG 73.4 Stage IV chest radiograph of fibrocystic pulmonary nose, nasal alae, and malar areas and the area around the eyes.
sarcoidosis with typical upward hilar retraction and multiple cystic Cardiac Sarcoidosis
and bullous changes.
Cardiac involvement is clinically apparent in <5–10% of patients
with sarcoidosis in North America and Europe, although autopsy
studies have suggested that the prevalence may exceed 25%. In
Japan, cardiac involvement is more common, occurring in more
than 50% of patients. Complete heart block, bundle branch
blocks, ventricular arrhythmias, sudden death, cardiomyopathy,
supraventricular arrhythmias, and valvular dysfunction may
occur. Current advice is to screen for cardiac sarcoidosis at
presentation, via assessment of symptoms (palpitations, syncope),
electrocardiography (ECG), and echocardiography. 45
Hepatic Sarcoidosis
Symptoms of hepatic sarcoidosis include fever, tender hepato-
megaly, and/or pruritus. Characteristically, serum alkaline
phosphatase and γ-glutamyl transferase are elevated proportion-
ately higher than aspartate and alanine aminotransferases or
bilirubin, although all patterns can occur. “Abdominal sarcoidosis”
refers to a constellation of liver, spleen, and abdominal lymph
node involvement, which is often associated with hypercalcemia;
FIG 73.5 Computed tomography scan demonstrating bilateral pulmonary involvement may not be evident.
hilar, paratracheal, and paraaortic lymphadenopathy with interstitial
infiltrates in a dominantly central bronchovascular distribution. Joints and Bones
Arthralgias are common in multisystem sarcoidosis, although
Pulmonary hypertension is an underrecognized complication joint radiographs are usually normal. Acute, often incapacitating,
of pulmonary sarcoidosis affecting at least 5% patients and up polyarthritis involving the ankles, feet, knees, and wrists may
to 50% of patients with dyspnea that is not explained by the occur in Löfgren syndrome. Persistent joint disease with pain,
44
level of pulmonary function impairment. Screening for pul- swelling, and tenderness of the phalanges of the hands and feet
monary hypertension is indicated in patients with advanced lung is found in <5% of patients with chronic sarcoidosis. Joint
disease because of the associated increased mortality. radiographs may demonstrate “punched-out” lesions with cystic