Friday, 2 September 2011

Symptoms of Sarcoidosis

Signs and symptoms of sarcoidosis                                   Image via Wikipedia
NB: This article is sourced from Chronic Illness Recovery. Please take a moment to visit their web site for detailed information about Sarcoidosis. 


Sarcoidosis is often referred to as a masquerader or the ‘snowflake’ disease because patients present with so many different symptoms. The clinical picture can be complicated by intermittent inflammation in many different organs, making diagnosis difficult.

While the symptoms may vary from patient to patient, the underlying cause is the same: intracellular bacteria


Inflammation therapy is designed to eliminate intracellular bacteria and effect recovery from sarcoidosis. 


The most common sarcoidosis symptoms listed by the Cleveland Clinic are:
  • Tender reddish bumps or patches on the skin
  • Red and teary eyes or blurred vision
  • Swollen and painful joints
  • Enlarged and tender lymph glands in the neck, armpits and groin
  • Enlarged lymph glands in the chest and around the lungs
  • Nasal stuffiness and/or hoarse voice
  • Pain in the hands, feet or other bony areas
  • Kidney stone formation
  • Enlarged liver (or spleen)
  • Development of abnormal or missed heartbeats (arrhythmias), inflammation of the covering of the heart (pericarditis) or heart failure
  • Nervous system effects, including hearing loss, meningitis, seizures or psychiatric disorders (for example, dementia, depression, psychosis)
There are many other symptoms that may be due to sarcoidosis, because it can damage or alter the function of any body organ.

Elevated 1,25-D
Sarcoidosis always results in dysregulated vitamin D metabolism and abnormally high levels of dihydoxyvitamin-D (1,25-D), a powerful hormone which affects many other hormones.

Symptoms of too much vitamin D (hypervitaminosis-D) in inflammatory disease may cause may adverse symptoms including:
  • Calcium deposits in the kidneys, lungs, blood vessels, heart and other soft tissues
  • High blood calcium levels
  • Irregular heartbeat
  • High blood pressure
  • High cholesterol

Elevated serum calcium
Approximately 6% of sarcoidosis patients have elevated serum calcium (hypercalcemia). This abnormal calcium homeostasis is not simply caused by increased gut absorption of calcium. Elevated 1,25-dihydroxyvitamin D3 stimulates intestinal calcium absorption and resorption of calcium and phosphates from bone. Calcium levels are normally regulated by the parathyroid hormone.

Excess amounts of 1,25 Dihydroxyvitamin D3 can result in bone resorption.

Clinical impact of bone and calcium metabolism changes in sarcoidosis

Precipitation of hypercalcaemia in sarcoidosis by foreign sun holidays: report of four cases.

A case of severe hypercalcemia with acute renal failure in sarcoidosis: a diagnostic challenge for the emergency department.

Light sensitivity and dietary vitamin D sensitivity
Sensitivity to sunlight was detailed in 1950 by Scadding and in 1954 by Anderson. In 1968 a paper by Winnacker, et al, reported that sarcoidosis patients can be 20 times as sensitive to sunlight and dietary Vitamin D as the 'normal' population. In fact, only 9000 IU of Vitamin D was a toxic dose to some sarcoidosis patients, whereas 'normal' people could take 150,000 IU without ill effect. Scadding (in 1950) reported that some sarcoidosis patients were intolerant of any dosage of Vitamin D.

The Canadian Lung Association warns sarcoidosis patients to stay out of the sun.

Exposure to excessive sunlight can make a sarcoidosis patient feel very ill, with symptoms that include fatigue, drowsiness, pain, "brain fog," memory problems, insomnia, sensitivity to light, visual problems, tingling and numbness, headache and nausea.

Someone who is exposed to excessive sunlight (such as on a vacation or moving to a sunnier climate) may have a symptom exacerbation that leads to a doctor visit and a diagnosis. This doesn't mean that the sunlight caused the sarcoidosis…it just made it more apparent.


Lungs
Patients with sarcoidosis may have some form of pulmonary involvement with the most common symptoms being a chronic cough and shortness of breath.
A chest x-ray or CT scan may reveal enlarged lymph nodes. Lymph nodes play a critical role in removing bacteria, abnormal cells and other matter as part of the immune system. A mediastinoscopy biopsy may be ordered to rule out cancer but enlarged lymph nodes are usually a sign of infection. The clinical picture may be enough to diagnosis sarcoidosis and an invasive biopsy isn’t necessary to treat the underlying chronic infection with inflammation therapy. See Diagnosing Sarcoidosis.

In later stages of sarcoidosis lymph nodes may become smaller, calcified and nonfunctioning.

X-ray images may also have the appearance of tuberculosis.

Lung involvement may be misdiagnosed as respiratory infection and must be differentiated from 'pneumonia'.

Sarcoidosis is considered a restrictive lung disease but it can cause pulmonary function test results to indicate obstructive lung disease, because chest lymph nodes may become so enlarged they compress airways, and/or granulomas themselves may obstruct airways.

Sarcoidosis can cause alveolitis, inflammation of the tiny sac-like lung air spaces where the exchange of carbon dioxide and oxygen take place. If the alveoli fill with fluid, there is less exchange of gases, which results in shortness of breath.

Because sarcoidosis causes interstitial inflammation it can result in crackles (rales) which are discontinuous, nonmusical, brief sounds heard more commonly on inspiration.

Sarcoidosis can result in bronchiectasis due to bronchial obstructions from enlarged chest lymph nodes or granulomas. Bronchiectasis is the abnormal widening of bronchi damaged by infection and the resulting inflammatory cytokines, or obstruction or traction. Traction bronchiectasis can occur when there is pulmonary fibrosis that distorts the airway.

Bronchiectasis can result in symptoms and signs that are uncommon in sarcoidosis - hemoptysis, recurrent infection, lung crackles and digital clubbing.
Clinical bronchiectasis complicating pulmonary sarcoidosis: case series of seven patients

Although rare, atelectasis (collapse of a lung or a portion of one, due to incomplete filling with air) can occur due to sarcoidosis because fibrosis can block an airway, leading to collapse of the alveoli.

A chest x-ray may reveal hyperinflation, sometimes called ‘air trapping’, causing shortness of breath. Impaired respiratory function may also be due to inflammation of nerves that supply respiratory muscles.

Pulmonary hypertension (PH) is a serious condition that may develop as a result of sarcoidosis. PH is a form of high blood pressure in the pulmonary artery, which connects the heart to the lungs.



Fatigue
One of the most frequent and universal symptoms of sarcoidosis is fatigue that is not relieved by sleep. The relentless fatigue of sarcoidosis may be discounted by medical professionals because "everyone gets tired", and there is no good way to measure fatigue.

Cognition
Short-term memory loss, poor concentration and ‘brain fog’ are common symptoms of sarcoidosis.

Skin
Cutaneous manifestations of sarcoidosis are varied and common. A patient can have different types of sarcoidosis skin lesions at the same time. Sarcoidosis is a dermatologic masquerader so physicians should be alert to investigating skin lesions and rashes.

This article by a practicing dermatologist explains that both "specific" and "non-specific" skin lesions are found in sarcoidosis.

Some of the types of skin lesions encountered in sarcoidosis are:
  • Papules (little bumps) resembling
  • Granulomatous rosacea
  • Acne
  • Benign appendageal tumors
  • Skin tags
  • Plaques resembling:
    -Psoriasis
    -Lichen planus
    -Nummular eczema
    -Discoid lupus
    -erythematosus
    -Granuloma annulare
    -Cutaneous T-cell lymphoma
    -Kaposi's sarcoma
    -Secondary syphilis
  • Lupus pernio resembling:
    -Scar tissue
    -Discoid lupus erythematosus
  • Erythema nodosum resembling
    -Cellulitis
    -Furunculosis
    -Other inflammatory panniculitis
Lupus pernio is said to be the skin lesion most characteristic (a diagnostic indicator) of sarcoidosis, but not all sarcoidosis patients have these skin lesions. Lupus Pernio is not another disease (and is not related to lupus), it's simply a name for the red-to purplish lesions that can appear on the nose, face, ears or hands of sarcoidosis patients. These lesions can occur when the nose, face or hands are exposed to cold or wind, so it's a good idea for patients to keep these areas protected from wind and cold. Avoiding exposure to natural light is also an important preventative measure.

Sometimes lupus pernio lesions will have an appearance of small "beads" along their edge, especially if the sore is on the rim of the nose. Lupus pernio lesions can be disfiguring and cause a patient to feel embarrassed, particularly since they may be noticeable on facial areas so visible to the public.

Like some other cutaneous sarcoidosis lesions, lupus pernio can appear at sites of old scars or trauma.

Lupus pernio lesions, like other sarcoid manifestations in the upper respiratory tract, can be quite resistant to the standard immunosuppressive therapies.

”My experience with lupus pernio is that it flares with exposure to light plus cold and wind. Warning of the risk of scarring, whether on the skin or in the lungs, is a common tactic to pressure patients to use the treatment a physician prefers. Lupus pernio has a reputation for being difficult to treat. In reality, the doctor has no way to know whether a patient will scar. Certainly I sustained no scars from my winter bouts with lupus pernio.” Belinda Fenter

Erythema nodosum is a common manifestation of sarcoidosis.

Subcutaneous nodular sarcoidosis lesions are sometimes called Darier-Roussy sarcoidosis, named for the French physicians Darier and Roussy who described these subcutaneous nodules in 1904.

Nails
Sarcoidosis can cause oncholysis (loosening or lifting of the nail at the border).

See:
Derm Net NZ (with photos)

Click here, to see a list of four PubMed-indexed articles about sarcoidosis nail disorders.

IMAGES OF CUTANEOUS LESIONS

Hair Loss and Plaquelike Skin Lesions in sarcoidosis (click on Figures 1, 2 and 3 in the article)

Insomnia is a common complaint by sarcoidosis sufferers.

Sleep apnea is the temporary absence of breathing during sleep. Patients with sarcoidosis are often diagnosed with disordered breathing (or sleep apnea).

Sarcoidosis can cause obstructive sleep apnea due to the fact that sarcoid inflammation and granulomas (which are tumors) can obstruct upper airway passages. In addition, use of prednisone for relieving sarcoidosis symptoms often results in significant weight gain and obesity has been related to sleep apnea.

According to the following article, sarcoidosis patients have a higher incidence of sleep apnea than the general population.


Pain
Joint, headache and muscle pain are common complaints and fibromyalgia, osteoarthritis, rheumatoid arthritis, migraine are common diagnoses associated with sarcoidosis.

Nose and sinuses
Biopsy of oral lesions often leads to a diagnosis of sarcoidosis. Sores or swelling can occur on the face, tongue, mouth, gums or inside the cheeks.

Orofacial Manifestations and Systemic Sarcoidosis

Facial Swelling and Systemic Sarcoidosis

Oral manifestations of sarcoidosis.

Granulomatous cheilitis (lip swelling) can lead to a sarcoidosis diagnosis

Maxilla
Sarcoidosis with maxillary involvement.

Saliva

Affected salivary glands can produce too much saliva or (more commonly) too little saliva. Too little saliva when inflammation impairs or blocks the function of the salivary glands can result in gum disease and increased dental cavities.

Sarcoidosis with Involvement of the Salivary and Lacrimal Glands

Gingivitis
Gingivitis is an inflammation of the gums and sarcoidosis is an inflammatory disease.


Dysphagia
It’s not unusual for people with sarcoidosis to experience
dysphagia (difficulty swallowing) as a symptom.

Dysphonia
Patients may also experience dysphonia, trouble with the voice when trying to talk, including hoarseness and change in pitch or quality or voice.

Dental fractures
Since sarcoidosis causes a dysregulation of Hormone D (1,25-dihydroxyvitamin D), a condition which causes calcium to be pulled from bones and teeth, the teeth can become weakened and susceptible to cracking. Sarcoidosis patients may experience multiple non-traumatic tooth fractures.

Pulp stones
Calcium pulled from bones and teeth is carried by the blood and deposited in soft tissues like the lungs, kidneys or dental pulp. Dental stones (pulp stones) can result from calcium deposits within the dental pulp. These may be found when root canal therapy is performed.

Eyes
Dry eyes, tearing, blurry vision
In the eye, the lacrimal gland produce tears to moisten and protect the eyes. It's not unusual for sarcoid patients to have uncontrolled tearing of the eyes, dry eyes or blurry vision. Lacrimal glands are a common area of manifestation for sarcoidosis.

Lid swelling and diplopia as presenting features of orbital sarcoid.

Cataracts
Sarcoid inflammation of the eye, uveitis or treatment with prednisone can result in a cataract.

Uveitis
Uveitis is inflammation of the eye structure called the uvea. Other parts of the eye can also become inflamed, causing conditions such as scleritis or iritis. Researchers found cell-wall deficient bacteria in the vitreous fluid of patients with sarcoidosis. Complications of uveitis may include glaucoma, cataract, abnormal blood vessel growth, fluid within the retina and vision loss.


Lymph Nodes
Sarcoidosis can result in systemic lymphadenopathy, including the abdomen.
Extensive abdominal lymphadenopathy in sarcoidosis.

Anemia
Anemia may be associated with sarcoidosis. Anemia of chronic disease is not due to iron deficiency and will not be helped by iron supplements.

Depression
Depression is commonly associated with sarcoidosis and may be due to expression of cytokines and other molecules usually associated with immune function.

Liver
Sarcoidosis may affect the liver, although it may be subclinical. An article from Medscape Gastroenterology  says: "The prevalence of hepatic granulomas in sarcoidosis is 65%. In a study of 100 patients with hepatic sarcoidosis, the majority of patients were asymptomatic and had normal abdominal examinations. Abdominal pain and hepatosplenomegaly were seen in 15% and 8% of these patients, respectively. Much less common were features of chronic liver disease and cirrhosis."

Extrapulmonary sarcoidosis primarily diagnosed in the liver.

Sarcoidosis of the liver

Patients may be told they have "fatty liver disease" or cirrhosis.

Pancreas
Asymptomatic organ involvement is quite common in sarcoidosis, including the kidneys. A case of renal sarcoidosis: a special reference to calcium metabolism as a diagnostic and the therapeutic implications.

Heart
Chest pain is a common symptom endured by sarcoidosis patients, substantiated by reports such as this one,which found that about 30% of sarcoidosis patients suffer from chest pain. 20 to 30 percent of sarcodiosis patients are found to have cardiac involvement upon autopsy.

The following study found that perhaps 50% of sarcoidosis patients have cardiac involvement that is not diagnosed. 

While it’s true that chest pain can be due to cardiac involvement, it may be due to other problems caused by sarcoidosis such as enlarged lymph nodes which can cause pressure, crowding and pain in the chest.

Muscles

Nerves
Neurological manifestations of sarcoidosis include:
  • Cranial nerve involvement
  • Impaired taste and smell
  • Blindness, blurry vision, double vision, visual field defects, pupillary abnormalities, dry and sore eyes
  • Facial droop, slurred speech, impaired swallowing, hoarseness
  • Vertigo, sensorineural deafness, tinnitus
  • Weakness of trapezius and sternocleidomastoid muscles
  • Tongue deviation and atrophy
  • Meningeal involvement
  • Headache is a prominent feature
  • Aseptic meningitis (mononuclear pleocytosis with increased protein)
  • Hydrocephalus (secondary to granuloma obstructing cerebral aqueduct)
  • Intraparenchymal involvement
  • Hypothalamus/pituitary disruption (electrolyte imbalance, changes in appetite, temperature, sleep and libido, diabetes insipidus, and syndrome of inappropriate secretion of antidiuretic hormone)
  • Diffuse encephalopathy (delirium, psychiatric disorders, cognitive impairment)
  • Seizure disorder (secondary to mass lesion, hydrocephalus, encephalitis, hypercalcemia)
  • Spinal cord involvement
  • Intra-, extramedullary; intra-, extradural process (myelopathy)
  • Peripheral nerve involvement
  • Mononeuropathy, mononeuropathy multiplex, polyneuropathy
  • Sensory neuropathy - characterized by loss of sensation or abnormal sensation (eg, tingling, numbness, painful patches over the thorax)
  • Motor neuropathy - characterized by weakness
  • Muscle involvement
  • Usually asymptomatic
  • Symptomatic myopathy seen in less than 8 % of systemic sarcoidosis patients
  • (May observe elevated creatine kinase)
[Neurosarcoidosis] (Yes, they sometimes want a brain tissue biopsy which isn't necessary)

Bell's palsy is a form of facial paralysis that occurs with damage to the nerve that controls movement of the muscles in the face. Bell's palsy may occur as a result of elevated 1,25-dihydroxyvitamin-D which is associated with sarcoidosis.

Heerfordt's syndrome is a cranial neuropathy (affecting mostly the facial nerve) with uveitis, parotid-gland enlargement, and fever. The syndrome is highly suggestive of sarcoidosis.

Horner syndrome, Argyll-Robertson pupil, and Adie's pupil have also been described in cases of sarcoidosis.

Sjögren's (sicca) syndrome
Sjögren's and sarcoidosis are both described in the standard literature as a collection of symptoms. Sjögren's is known to be related to diseases such as rheumatoid arthritis, sarcoidosis, lupus, scleroderma and polymyositis. Many sarcoidosis patients manifest the same symptoms as described in Sjögren's syndrome (dry eyes and mouth, decreased tears and saliva, and resulting dental caries).

Infertility
Sarcoidosis inflammation may affect the reproductive organs such as the testicles and cause temporary infertility.


Last updated January 16, 2011


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