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Sarcoidosis or Sarcoid : Definition, Causes, Risk Factors, Symptoms, Signs, Diagnosis, Treatment

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Sarcoidosis is a Greek word means “Resembling Condition”. Sarcoidosis is an inflammatory disease spread in several organs in human body. Inflammation causes granuloma formation, which is known as Sarcoid. Sarcoidosis or Sarcoid usually affects lungs and skin. The exact cause for Sarcoidosis or Sarcoid is still not yet known. In this article, we will discuss in detail about the causes, symptoms, and treatments rendered for Sarcoidosis or Sarcoid.

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Sarcoidosis or Sarcoid
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How Is Sarcoidosis or Sarcoid Defined?

Inflammatory Disease- Sarcoidosis or Sarcoid is a inflammatory disease that consists of granuloma.1

Wide Spread Disease- Disease is wide spread in multiple organs.

Relapse-

  • The sarcoidosis disease tends to come and go all of a sudden.
  • Disease may progressively develop as a serious illness.
  • Patient may experience several relapse throughout the life.

Granulomas-

  • Sarcoidosis or Sarcoid is a gradual progressive disease.
  • Microscopic lumps called granulomas start to appear in the affected organs.1
  • In most of the cases, these granulomas tend to clear with or without treatments.
  • There are few instances where granuloma grows in size and continues to be a part of the organ.
  • Granuloma eventually ends up as fibrotic lump but may cause several complications.

Organs Involved in Sarcoidosis or Sarcoid-

Organs Involved in Sarcoidosis or Sarcoid

  • Respiratory system- trachea, bronchi and Lungs,2
  • Skin and subcutaneous tissue,
  • Liver- causes hepatomegaly (enlarged liver)3
  • Heart– causes arrhythmia
  • Lymphatic system- causes enlarge lymph nodes and Splenomegaly (enlarged spleen)4
  • Brain– causes granuloma of the brain resulting in confusion, hallucination and unconsciousness.5

Hypercalciuria (Increased Serum Calcium Level)

  • Causes renal stone
  • Serum calcium level is high secondary to abnormal calcium metabolism caused by increased secretion of 1, 25 dihydroxyvitamin D.
  • 1, 25 dihydroxyvitamin D secreted by macrophages, which lies in sarcoid granuloma.

Causes of Sarcoidosis or Sarcoid

The definitive cause of Sarcoidosis or Sarcoid is not known.

  • Autoimmune Disease-
    • Literature and research suggests Sarcoidosis or Sarcoid is caused by abnormal immune activities.
    • Viral infection, chemicals or drugs trigger immune reaction.
    • Antibodies secreted by the body causes inflammatory tissue reaction and formation of granuloma.

Risk Factors For Sarcoidosis or Sarcoid

  • Frequency-
    • Sarcoidosis or Sarcoid is quite a common medical condition and occurs practically throughout the world.
  • Age-
    • Sarcoidosis or Sarcoid often occurs between the ages of 20 and 40.
  • Sex-
    • Women are slightly more likely to develop the disease.
  • Race-
    • African-Americans have a higher incidence of Sarcoidosis or Sarcoid than Caucasian.
    • Recurrence of Sarcoidosis may be more severe and likely to recur in African-Americans.
  • Family History
    • If someone in your family has had Sarcoidosis, you are more likely to develop the disease yourself.

Signs and Symptoms Of Sarcoidosis or Sarcoid

Non-Specific Symptoms Of Sarcoidosis or Sarcoid-

Specific Symptoms Of Sarcoidosis or Sarcoid-

  • Sarcoidosis or Sarcoid Of Lungs, Trachea And Bronchi2
    • Chest pain
    • Wheezing
    • Dyspnea- Shortness of breath
    • Chronic cough
    • Granuloma lesion of bronchi or trachea may cause obstruction to breathing.
  • Sarcoidosis or Sarcoid of Skin
    • Skin rash- spread over face, arms and shins.
    • Skin lump- mostly observed over back, arms, legs and scalp
    • Disfiguring sores- seen over nose, nasal passage, ears, eyelids and fingers
    • Skin ulcer- mostly observed over back, arms and legs.
  • Sarcoidosis or Sarcoid of Eyes-seen in 25% of cases.
    • Anterior and posterior uveitis
      • Redness
      • Pain- burning and stabbing type
      • Photophobia and blurry vision
      • Progressive loss of vision
    • Lacrimal gland swelling
      • Itching
      • Pain
  • Bones and Arthralgia-
    • Joint Pain is observed in 30% of the cases.
    • Bone lesions (cysts)- cystic and sarcoid lesions are observed in 5 to 10% of patients.
  • Lymphatic System
    • Enlarged lymph node- Neck, Thoracic, Throat, Armpit and Groin
    • Splenomegaly4
  • Renal Stone-
    • Severe colic pain in flank
    • Caused by Hypercalcemia (increased blood calcium)
    • Seen in 10% of Sarcoidosis or Sarcoid cases
  • Renal Failure- Granulomatous Interstitial Nephritis
    • Flank pain
    • Retention of fluid
    • Peripheral edema
    • Pleural effusion
    • Hepatomegaly
  • Nervous System- Granulomatous Lesion5
    • Brain-
    • Cranial Nerve-
    • Peripheral Nerves-
      • Sensory neuropathy
      • Muscle weakness
    • Cognitive Disorder-
      • Psychosis
  • Cardiovascular Effects-
    • Vascular effects-
      • Hypertension
    • Cardiomyopathy
      • Irregular heart beats
      • Arrhythmia
      • Palpitation
      • Sudden death

Signs of Sarcoidosis or Sarcoid-6

  • Hepatomegaly- enlarged liver
  • Splenomegaly- enlarged spleen
  • Arrhythmia- abnormal heart beats
  • Hypertension
  • Wheezing during breathing

Diagnosis of Sarcoidosis or Sarcoid

Urine Examination

  • Hypercalceuria
  • Proteinuria

Blood Examination

  • Serum calcium level- high (Hypercalcemia)
  • Lymphopenia- low lymphocyte count
  • Hemolytic Anemia- low hemoglobin
  • Leukopenia– white blood cell count low
  • Thrombocytopenia- platelet count low
  • Alkaline Phosphatase- elevated

Ultrasound

  • Ultrasound findings are as follows-
    • Hepatomegaly- enlarged liver
    • Splenomegaly- enlarged spleen
    • Lymphadenopathy- enlarge lymph nodes

X-Ray Chest

  • Routine X-Ray may show Hilar and Mediastinal sarcoid granuloma as a radio opaque shadow.
  • Further investigation is necessary.

CT Scan or MRI Study-

  • Lungs-
    • Bilateral Hilar Lymphadenopathy (BHL)- observed in 95% of asymptomatic individuals.
    • Findings should be confirmed with biopsy
  • Brain-
    • Swelling of brain suggest encephalogram
  • Liver-
    • Enlarged liver (Hepatomegaly)
  • Kidney-
    • Interstitial granulomatous nephritis
  • Heart-
    • Cardiomyopathy

Biopsy Procedures-

Findings of Biopsy- Biopsy of Sarcoidosis or Sarcoid indicate 90% of white blood cells are Lymphocytes. In few cases normal percentage of eosinophils and neutrophils are seen in granulomatous tissue.

  • Tissue Biopsy-
    • Peripheral lymph nodes
    • Hilar or chest lymph node
    • Skin granuloma
    • Nasal mucosa
  • Bronchial Mucosal Biopsy
    • Done to rule out lung or bronchial carcinoma, when Hilar lymph node are observed during radiological study.
  • Lung Biopsy
    • Biopsy of lung granuloma tissue
    • Plasma cells absent
    • Foamy alveolar macrophages are present

Electrocardiogram and Echocardiogram Test –

  • Arrhythmia and irregular heartbeats are observed

Ophthalmoscope Examination

  • Increase eye pressure- suggest glaucoma
  • Uveitis

Treatments For Sarcoidosis or Sarcoid

Anti-inflammatory

  • NSAIDs- indicated for inflammation.

Immunosuppressant

Indicated for autoimmune effects of Sarcoidosis.

  • Corticosteroids
  • Steroid sparing agents-
    • Methotrexate
    • Azathioprine
    • Mycophenolic acid
    • Leflunomidel
    • Cyclophosphamide

Hypertension

  • Ace inhibitors-
    • Prescribed for hypertension
    • Protects kidney when diagnosis is Sarcoidosis or Sarcoid of kidney.

Antimalarial Medications

  • Prescribed for Cutaneous Sarcoidosis
  • Chloroquine and hydroxychloroquine found to be effective in treating Sarcoidosis

Phosphodiesterase 4 inhibitors

  • Experimental medication inhibits phosphodiestrase 4.
  • Roflumilast
  • PDE4 inhibitors
  • Pentoxifylline

Quercetin-

  • Tried in research trial
  • Response to treatment- excellent.

References:

  1. Sarcoids.
    Bergvall KE.
    Vet Clin North Am Equine Pract. 2013 Dec;29(3):657-71. doi: 10.1016/j.cveq.2013.09.002.
  2. Pulmonary sarcoidosis: diagnostic and treatment update.
    Fahim A1, Mann JS.ar, Z. Medscape Reference. W
    Expert Rev Respir Med. 2014 May 16:1-9.
  3. Isolated hepatic sarcoidosis.
    Jovicić I, Popović DDj, Toncev L, Jovicić Z, Vucnić V, Kovacević N, Djuranović S, Boricić I, Micev M, Spuran M, Milosavljević T, Mosenif.
    Vojnosanit Pregl. 2014 Apr;71(4):399-403.
  4. Laparoscopic splenectomy for isolated splenic sarcoidosis.
    Souto MM, Tempes BC, Lambert BF, Trindade EN, Trindade MR.
    SLS. 2014 Jan-Mar;18(1):155-9.
  5. Neurologic manifestations of sarcoidosis
    Krumholz A1, Stern BJ2.
    Handb Clin Neurol. 2014;119:305-33.
  6. Sarcoidosis.
    Valeyre D1, Prasse A2, Nunes H3, Uzunhan Y3, Brillet PY4, Müller-Quernheim J2.
    Lancet. 2014 Mar 29;383(9923):1155-67.
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Team PainAssist
Team PainAssist
Written, Edited or Reviewed By: Team PainAssist, Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:February 3, 2023

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