Sarcoidosis 4.54/5 (37)

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Sarcoidosis: Swollen lymph nodes in both lungs. Illustration: Mikael Häggström / Wikimedia Commons. CC0 1.0

Sarcoidosis (ICD-10 D86) and Löfgren's syndrome

Definition

Sarcoidosis is a chronic disease in which inflammatory cells of unknown origin form nodules in the tissue in the form of granulomas with enlarged lymph nodes and changes in other organs as well.

  • Lung changes are best known, but sarcoidosis in the skin, joints, heart and eyes occurs in approximately 30%

The disease can develop over the course of days to weeks or manifest itself gradually over months to years. Some people do not notice any symptoms and the disease is detected randomly by x-ray examination of the lungs. Most often, sarcoidosis is harmless and goes away on its own, but some become seriously ill and need bowing. In the early stages of the disease, enlarged lymph nodes can be detected in the lungs by x-ray or CT examinations. The diagnosis is preferably guaranteed by tissue test (biopsy). Sarcoidosis is not considered among the typical connective tissue disorders, but has several common symptoms. Norwegian dermatologist Caesar Boeck described the skin symptoms of sarcoidosis in 1899.

Occurrence

Sarcoidosis is usually developed before the age of 50 and has been detected in all population groups. Women are attacked about twice as frequently as men. However, the prevalence varies between geographical areas, families and ethnicity. The disease is more common in Scandinavia than in the world.

  • Swedish data indicate that 12 people get the disease per 100,000 population per year (incidence), which corresponds to about 600 new cases in Norway annually and that between 7500 and 10.000 people have the disease (reference: Arkema EV, 2016)

Symptoms

General condition / general symptoms

  • Feeling of exhaustion (66%). Fatigue, fever, night sweats and weight loss

Bone marrow

  • Occasionally the bone marrow is attacked, resulting in reduced blood cell count (low hemoglobin, low white blood cell count and platelet count)

Heart

House: Erythema nodosum can be sarcoidosis. Erik H www.gnu.org/copyleft/fdl.html) or via Wikimedia Commons. CC BY SA 3.0

Sarcoidosis: Lupus pernio. M. Sand, D. creativecommons.org/Wikimedia Commons CC BY 2.0

Skin

About 25% get skin symptoms that are a little more common among women.

  • At about 10% with sarcoidosis, blue-red, slightly tender thickening occurs in and under the skin on the front of the leg or elsewhere ("knot-rose" = (Erythema nodosum) in early disease phase. Changes can vary in size up to a few centimeters in diameter and alternate between gradually decreasing over a few days and appearing in new places. Within a few weeks (usually 3 weeks) to months, the symptoms go on by themselves. Tissue sample of erythema nodosum shows panniculitis, but without evidence of sarcoidosis.
  • Other types of skin symptoms also occur: Small red spots or knots, often on the face or on old scars are common.
  • Chronic sarcoidosis is characterized by a distinct red-blue, psoriasis-like eczema. Some get the so-called “Lupus pernio"Eczema which is violet discolored areas of the nose, cheek or ears. It is due to sarcoidosis and not lupus disease (SLE).
  • Some with sarcoidosis in the skin needs long-term treatment and follow-up

Sarcoidosis in the eye, joints and skin. Sharma SK - Indian J. Med. Res. (2012). CC BY-NC-SA 3.0

Joints and muscle manifestations

Løfgren Syndrome

  • Between 9 and 34% with sarcoidosis, Løfgren's syndrome (Reference: Siltzback LE, 1974)
  • Acute joints that involve swelling of the ankles and surrounding skin
  • Erythema nodosum
  • Enlarged lymph nodes in the lungs (see Lungs above)
  • Also knees can swell
  • In the case of typical Løfgren`s syndrome, sarcoidosis is the most likely diagnosis that tissue testing (biopsy) is often considered unnecessary
  • The syndrome is self-limiting. The skin changes are most often over by themselves after a few months, while the arthritis lasts longer, often about two years

Rarely, chronic arthritis occurs in small joints (0,9%), especially finger-like arthritis (Rheumatoid arthritis).

Some get thickening throughout the length of the fingers ("sausage fingers" / dactylitis)

Liver

  • Enlarged Liver (hepatomegaly) seen at 5-15% by CT examination. Changes can be detected more if tissue samples are taken
  • Blood tests often show slightly elevated liver enzymes, usually alkaline phosphatase (ALP)
  • Most people with sarcoidosis in the liver also have general symptoms such as night sweats, fever tendency, fatigue and weight loss

Lungs

More than 90% have lung manifestation

Lymph nodes

Sarcoidosis occurs in lymph nodes in different parts of the body

  • Lymph nodes swell in approximately 15%

Spleen

Large spleen (splenomegaly) occurs at approximately 25%

Muscle Inflammation

Myositis is a rare complication, but can cause muscle weakness and is detected by tissue testing

The nervous system

About 10% with sarcoidosis have symptoms of the nervous system

  • The brain can be affected early in the course of the disease, while peripheral nerves are most often attacked later
  • MRI examination of the brain and lumbar puncture with assessment of CSF contributes to the diagnosis

Hormone changes (from pituitary gland)

  • May occur without obvious sarcoidosis in other organs

Kidneys

  • Increased absorption of calcium from the intestine can lead to elevated blood calcium levels (in 10-20%) and high calcium in urine (in 50%).
  • Some then develop calcium stones in the kidneys (kidney stones). The cause is also abnormally high production (in activated macrophages) of 1,25-dihydroxycholecalciferol (Vitamin D3) in sarcoidosis

Salivary glands

The eye

  • Tear glands can damage so that tear production becomes low. Dry eyes are therefore common in prolonged illness. About 5% has eye symptoms from the early stages of the disease and up to 20% can have it later in the course of the disease
  • Eyes can also be attacked with inflammation (without pus) caused by sarcoidosis. One senses pain when looking at the light, most often seen redness in the eye (Anterior uveitis, iridocyclite, keratoconjunctivitis), but also other forms of eye inflammation and vision impairment (chorio-retinitis, retinal vasculitis)
  • Women often have eye manifestations
  • Eye disease more detailed description of sarcoidosis here

Studies on Sarcoidosis

Symptoms of skin, joints and organs are being examined by a general practitioner or specialist for the organ that exhibits the most symptoms

  • X-ray examination or CT examination of lungs
    • More than 90% have typical changes seen on X-rays or CT examinations
  • By bronchoscopy (pulmonary doctor) the presence of various white blood cells in the respiratory tract can be analyzed. A CD4 / CD8 ratio of more than 3,5 speaks for sarcoidosis
  • Further investigation (tissue samples, X-ray examination of joints, MRI, PET / CT examinations) will depend on indication
  • MRI examination of the heart (special examination) can detect increased contrast agent uptake in parts of the heart muscle
  • ECG to evaluate cardiac arrhythmias
  • Blood tests
    • SR (lowering reaction) and CRP are often somewhat elevated
    • ACE (angiotensin converting enzyme) may be elevated in widespread disease
      • The reference area (adult 18-65 U / L, child 9-13 is up to 40% higher: about 20-100 U / L at OUS laboratory)
      • Elevated ACE is also seen in infection with mycobacteria, HIV and fungi, in diabetes mellitus, thyroid toxicosis, pulmonary fibrosis and severe pulmonary failure.
      • In sarcoidosis with erythema nodosum (and possibly arthritis in Løfgren's syndrome), ACE is usually normal
      • Treatment with ACE inhibitors (against high blood pressure) causes low ACE, but does not affect the disease
      • Low ACE is of no disease significance
    • Anti-CCP (ACPA) and other "rheuma tests" are expected to be normal
    • Immunoglobulin IgG is often low
    • Calcium may be elevated in blood and (more often) in urine

Diagnosis

A combination of clinical examination, image diagnostics and tissue testing ensures the correct diagnosis:

    1. Symptoms or imaging that fits with sarcoidosis
    2. Tissue sample with non-necrotizing granulomas
    3. Exclusion of other diseases with similar symptoms, examination findings and similar tissue test
  • In case of skin, joint, eye and internal organs, these are first examined by a general practitioner, then by a specialist for the organs most attacked
  • Blood samples with CRP, calcium
    • ACE ("angiotensin converting enzyme") produced by the granulomas is detected elevated in approximately 60% with sarcoidosis. (When using ACE inhibitors as a blood pressure drug, ACE is low in any case)
  • Investigation (tissue samples, X-ray of joints, MR, PET / CT) will depend on indication
    • PET / CT can provide a clear representation of the spread of the disease and show areas relevant to biopsy
    • Indication will be dependent on whether the result will be of therapeutic importance (Reference: Treglia G, 2014)
  • The diagnosis is ensured by biopsy (tissue sample). This can be taken from lung by ultrasound-controlled endo-bronchiocopy (EBUS). This is done by a lung doctor. Typically detected granulomas by microscopic examination. Tissue samples from the skin, bone marrow, muscles or liver may sometimes help to make the diagnosis
  • If a chronic joint infection is suspected, a rheumatologic examination is often supplemented by X-ray or MRI joint examination. A tissue sample from the joint (in the joint) can detect typical sarcoidosis changes (granulomas)
  • When examining other organ infection, tissue testing is important for diagnosis

Incorrect diagnosis? (Similar diseases / differential diagnoses)

Differential diagnoses of sarcoidosis are described in greater detail on their own here

Treatment of sarcoidosis

Most people with sarcoidosis need no special treatment. Inflammatory suppressing NSAIDs like Ibux are used against pain. One is reluctant to use Prednisone or other cortisone, since the condition is most often self-limiting and side effects can exceed utility. But if the general condition is heavily influenced by fever, weight loss, or severe pain, prednisolone may be useful. If multiple organs are attacked or chronic, multiple drugs may be combined. Among current drugs are:

In sarcoidosis in the heart and severe rhythm disorders can pacemakers and / or implants defibrillator (ICD) be necessary.

Follow-up of sarcoidosis

When sarcoidosis is to be followed up within the specialist health service, it is usually appropriate that it occurs within the specialty that knows the main manifestations best, for example

  • Pulmonary physician of the lungs
  • Ophthalmogist for eyes
  • Rheumatologist for musculoskeletal and skeletal disorders
  • Dermatologist for the skin
  • Cardiologist for the heart
  • Neurologist for the nerve system

There is no single blood test, but CRP, calcium and ACE ("angiotensin converting enzyme") are also in progress.

  • Lung manifestations can be followed by lung function tests and CT examinations if needed
  • Ultrasound, CT or MRI examinations of enlarged organs
  • PET / CT most often shows marked FDG recordings where the disease is active
  • Cardiac sarcoidosis can be followed with MRI of the heart, PET / CT and ECG examinations
  • If there is an indication for Prednisolone, starting doses of 20-40 mg / day at a lower dose are often chosen depending on the treatment response. One aims at treatment for at least one year. In relapse, the last effective dose is increased. If Prednisolone dose in the long term does not fall below 10-15 mg / day, additional immunosuppressive therapy is required (please see above)

Prognosis

The disease goes down by more than 50% after about three years from the time of diagnosis.

  • After ten years, two out of three are without signs of active sarcoidosis
    • In the absence of active illness for more than one year, relapse is unusual

Unfortunately, sarcoidosis leaves lasting damage in various organs in one of three.

  • Less than 5% die of sarcoidosis.
    • Causes of death are lung failure, heart disease or nervous system attack (Reference: Lannuzzi MC, 2007)

The referral

Medical Examination

Literature


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