Having discussed "Overview of Sarcoidosis" and "Skin Manifestations of Sarcoidosis", we will now continue to discuss other manifestations of sarcoidosis.
First, sarcoidosis affects the eyes
Data from Japan show that its prevalence is much lower than in Europe and the United States (see Overview of Sarcoidosis); however, patients with sarcoidosis in Japan are more likely to have eye and heart disease. We don't know if the characteristics of sarcoidosis patients in China are the same. But Japan's data is worth learning.
Up to 25% of patients with sarcoidosis have ocular involvement, and 5% of patients complain of ocular involvement; current reports have found that eye lesions are more common in women, African-American, and Japanese patients.
Sarcoidosis can affect the orbit, anterior and posterior segments, conjunctiva, uveal membranes, lacrimal glands, extraocular muscles, fundus, etc.;
A study from a sarcoidosis ophthalmic referral center found that 40% of those with ocular symptoms had uveitis, 30% had dry corneal conjunctivitis, and 15% had granulomas of ocular appendages (eyelids or conjunctiva). Symptoms include:
- Dry eyes,
- blurred vision,
- photophobia
- Redness
- ophthalmalgia
In patients with a definitive diagnosis of sarcoidosis, ophthalmologists are advised to examine the eye, even if there is no indication of ocular involvement. in order to detect underlying eye lesions, especially fundus;
These patients should have vision testing, intraocular pressure measurement, slit lamp examination, and fundus examination to assess ocular involvement; fluorescein fundus angiography may be helpful in finding retinal vascular abnormalities such as retinal vasculitis and perretinal venoleitis.
The diagnosis of ocular sarcoidosis should refer to the consensus guidelines of the International Workshop on Ocular Sarcoidosis (IWOS);
Anterior uveitis, parotid gland enlargement, facial nerve palsy (due to enlarged parotid gland compression), combined with fever, is called "uveal parotid fever", also known as Heerfordt syndrome. The syndrome is more specific and suggests sarcoidosis, but is rare.
Muttonoid corneal deposits, suggestive of sarcoidosis, reference 1
Second, the head and neck manifestations of sarcoidosis
Sarcoidosis can involve cervical lymph nodes, pharynx, larynx, nostrils, and/or sinuses. Cervical lymphadenopathy is the most common.
Other head and neck manifestations:
- Laryngeal sarcoidosis: dysphagia, dyspnea, cough, and hoarseness
- Rhino-sinus sarcoidosis: nasal congestion, crusting formation, loss of smell, nosebleeds, nasal races
It is important to distinguish from diseases such as tuberculosis, granulomatous polyangiitis, leprosy and syphilis.
Third, sarcoidosis affects the cardiovascular system
Autopsy studies show a 25% to 70% incidence of subclinical cardiac involvement. People with symptoms usually complain of palpitations, syncope, dizziness, chest pain.
The greatest risk of cardiac sarcoidosis is that it can lead to fatal arrhythmias. Some sudden deaths have been found to be due to cardiac sarcoidosis. It is not certain that sarcoidosis increases the risk of sudden death.
Initial evaluation of all patients with sarcoidosis should include an ECG. For patients with suspected cardiac involvement, the American Society of Thoracic Medicines consensus guidelines recommend cardiac magnetic resonance imaging (CMR) rather than cardiac PET (cPET) or transthoracic echocardiography. If the institution does not have the conditions to perform CMR, or if the CMR results are unclear, a specialized cPET (not TTE) should be performed to obtain diagnostic and prognostic information.
Of course, other tests can be scheduled based on symptoms, signs, and ECG findings. For example, 24-hour Holter monitoring and echocardiography.
Sarcoidosis can also cause pulmonary hypertension and pulmonary heart disease. Initial screening for pulmonary hypertension may do cardiac ultrasound; however, invasive pulmonary artery manometry is relied upon in highly suspected cases.
Fourth, lymph nodes, liver and spleen
Sarcoidosis predisposes to the lymph nodes, liver, and spleen.
1. Lymphadenopathy: up to 90% of patients can see hilar and/or paratracheal mediastinal lymphadenopathy in imaging examination; up to 40% of patients with sarcoidosis have peripheral lymphadenopathy. CT scan of the abdomen shows that about 30% of patients with sarcoidosis have intra-abdominal lymphadenopathy (there are 2 lymph nodes on the short axial surface>1 cm in diameter).
2, liver: patients with hepatic sarcoidosis are usually asymptomatic. However, abnormal liver function tests and hepatomegaly are common; a small number of patients may develop advanced liver disease. For sarcoidosis patients with no liver symptoms or a confirmed diagnosis of liver involvement, the Consensus Guidelines of the American Thoracic Society recommend a serum alkaline phosphatase test to screen for liver sarcoidosis. Transaminases may also be elevated, but not as prominent as elevated alkaline phosphatase.
On abdominal enhancement CT, typical manifestations are hepatomegaly and multiple 1 mm to 3 cm low-density nodular foci; T1-weighted images of MRI show low-signal nodular foci, while T2-weighted images show hyperinsity of nodules relative to the surrounding hepatic parenchyma; lesions are often located around the portal vein.
3, splenomegaly: quite a few patients have splenomegaly, but there are not many manifestations of hypersplenism (anemia, leukopenia, thrombocytopenia).
Sarcoidosis involves the liver; characterized by low-density shadows; from Reference 1
Fifth, nodular disease of the digestive tract
Oral sarcoidosis presents with nodules, edema, ulcers, or gingival abnormalities (eg, gingivitis, hypergingia, gingival retraction). The most commonly affected areas are the buccal mucosa, followed by the gums, lips, base/sublingual glands, tongue, and palate.
The esophagus may also be affected. The most common complaint is dysphagia; in addition, there is weight loss, abdominal pain, chest pain, etc. Endoscopic esophageal manifestations are nonspecific and include discontinuous gray plaque-like lesions with a diameter of 3–10 mm, as well as mucosal hyperemia and nodules.
Sarcoidosis can spread to the stomach. But usually there are no specific symptoms. Endoscopic manifestations of gastric sarcoidosis include nodular changes, erosive gastritis, thickening of the gastric mucosa, gastric curvature or gastric curvature deformation, and ulcers with benign or malignant appearance. Nodular mucosal irregularities are common; however, gastric mucosal polyps are uncommon.
Sarcoidosis of the small intestine is rare. The most common symptom of involvement of the small intestine is non-bloody diarrhea; epigastric and/or peri-umbilical colic in about one-second of cases; weight loss, anorexia, low-grade fever, and fatigue may also occur. Rare manifestations of small bowel nodular disease include: protein-losing bowel disease;
Sarcoidosis also rarely affects the colon and rectum; the most common symptom is abdominal pain, followed by diarrhea and weight loss; and megaloblastic anemia has also been reported.
In patients with diarrhea, colorectoscopy, or even duodenal, or microintestinal examination is usually preferred. When presenting with abdominal pain, CT is preferred.
(To be continued)
Resources:
1, Uptodate Clinical Consultant
2. Rheumatology (7th Edition)
3，《Firestein & Kelley’s Textbook of Rheumatology》（第 11 版）