Sarcoidosis Causes Diagnosis Symptoms and Treatment

Sarcoidosis

It is a chronic, multisystem disorder of unknown cause.

Sarcoidosis DiagnosisDyslipidemias Causes Risk Factors Epidemiology Etiology. Read more ... » Characterized by :

Organs most commonly involved:

Course of Disease may be

Sarcoidosis Etiology

  • · Cause unknown
  • · Self-antigens may be responsible for disease
  • · Disease is due to exaggerated cellular immune response.

Sex

  • · Females may be more susceptible
  • · Male: Female 1: 1, both sexes equally affected usually.

Age

  • · Common between 20 – 40 years
  • · Children and elderly can also be affected.

Sarcoidosis Pathophysiology and Immunopathogenes’s

  • · Accumulation of CD4 + TH11ymphocytes in or- gan
  • · Granulomas
  • Thought to be due to exaggerated cell-mediated immune response to unknown antigen(s)
  • In the lungs, the initial lesion is CD4+ T-cell alveolitis, causing noncaseating granulomata, which may resolve or undergo fibrosis.
  • · Multinucleated giant cells/Langhans giant cells
  • · Schaumann bodies-Conch-like (Shankh)
  • · Asteroid bodies.

Sarcoidosis Clinical symptoms

Constitutional symptoms are present:

Respiratory symptoms: CoughInfluenza SARS and Whooping Cough Signs and Symptoms with Diagnosis and Treatment. Read more ... », Dyspnoea

Retrosternal chest discomfort

Polyarthritis Lofgrens syndrome:

  • · Erythema nodosum
  • · Bilateral hilar adenopathy
  • · Arthritis of ankles, knees, wrists, elbows.

Heerfordt- Waldenstrom syndrome:

  • · Fever
  • · Parotid enlargement
  • · Anterior uveitis
  • · Facial nerve palsy.

Lungs

Necrotizing sarcoidal granulomatosis

Lymph node enlargement

  • · Discreet
  • · Firm
  • · Rubbery
  • · Painless.

Lymphadenopathy - (usually of hilar nodes)

  • · Paratracheal lymph node
  • · Mediastinal lymph node
  • · Cervical
  • · Axillary
  • · Inguinal
  • · Mesenteric
  • · Retroperitoneal.

Skin

  • · Erythema nodosum
  • · Plaques
  • · Subcutaneous nodules
  • · Maculopapular eruptions
  • · Lupus pernio – purple, swollen and shiny patches on nose, cheeks, lips, ears, fingers
  • · Clubbing of fingers
  • ·Polyarthralgias may occur with skin lesions. Resolves in 2 to 4 weeks.
  • Skin lesions are usually seen on face-around eyes and nose, buttocks, extremities, back.

Eyes

Keratoconjunctivitis sicca syndrome

  • • Dry, painful eyes.

Upper respiratory tract

  • · Nasal stuffiness
  • · Tonsil involvement
  • · Tongue involvement
  • ·Larynx.

Symptoms

  • · Hoarseness of voice
  • · Dyspnoea
  • · Wheezing
  • · Stridor
  • · Even complete obstruction of passage.

Bone marrow / Spleen

  • · Anaemia
  • · Neutropenia
  • · Eosinophilia
  • · Thrombocytopenia
  • · Splenomegaly.

Liver

Kidney

  • Rare involvement
  • Granulomas produce 1, 25 dihydroxy-vitamin D which can lead to increased calcium absorption in the gut causing nephrolithiasis.
  • Tubular, glomerular, renal artery disease can occur.

Nervous system

Musculoskeletal system

  • · Involvement of bones, joints, muscles
  • · Swelling of digits
  • · Arthritis
  • · Deformities of joints
  • · Polymyositis.

Heart

Endocrine and Reproductive system

Pregnancy

  • · Patient may improve during pregnancy but wors­ens after del ivery.

Parotids involvement

  • • Bilateral non-tender, smooth enlargement.

Gastrointestinal system (GIT)

  • • Rare,

Death

  • · Occurs due to respiratory disease
  • · CHF,

Diagnosis Test –

Sarcoidosis INVESTIGATIONS

  • Lymphocytopenia Increased ESR Hyperglobuli.nemia
  • Increased ACE (angiotensin converting enzyme) lev­els,

Chest x-ray

  • · Type I-
  • · Type II
  • bilateral hilar lymphadenopathy
  • - bilateral hilar lymphadenopathy with parenchymal changes
  • Type III – no hilar lymphadenopathy, only dif­fuse parenchymal changes
  • Type I - acute, reversible
  • Type Il, III - chronic, progressive

X-Ray findings

  • · Egg-shell calcification of lymph nodes
  • · Pleural effusion
  • · Cavitation
  • Collapse Pneumothorax Cardiomegaly
  • Pulmonary hypertension changes,

Lung function tests

  • · Decreased lung volumes
  • · Airflow limitation,
  • Gallium 67 lung scan
  • • Diffuse uptake.
  • Bronchoalveolar lavage (BAL)
    • • IncreasedCD4+THl.

Sarcoidosis TREATMENT

  • Therapy of choice is glucocorticoidsAdrenal Gland Steroids. Read more ... » : Prednisone­1 mg/kg for 4-6 weeks, tapered in 3 months.
  • Others are:
    • Methotrexate – 5-15 mg/week single dose orally.
    • Indomethacin
    • Chloroquine
    • Pentoxifylline
    • Allopurinol
    • Levamizole
    • Azothiaprine
    • Cyclophosphamide
    • Cyclospori ne,
  • There is spontaneous remission or cure in 50% of patients, However glucocorticoids may be given in symptomatic patients or patients with organ derange­ment,

First Line TREATMENT

Second Line TREATMENT

  • Azathioprine: 50–100 mg/d
  • Cyclophosphamide: 25–50 mg/d, increasing to goal white blood cell (WBC) count of 4,000–7,000/mm3
  • Hydroxychloroquine (Plaquenil): 100–400 mg/d
  • Infliximab, a chimeric monoclonal antibody, has been useful in refractory cases. Dose is 3–5 mg/kg IV initially, 2 weeks later, then q4–6wk.
  • Methotrexate: 10–15 mg/wk
  • Thalidomide has been used for chronic skin lesions. The anti-tumorUses of Ayurveda for Improvement in Cancer Patients Treatment. Read more ... » necrosis factor (TNF) agent infliximab also has been used in some refractory cases
  • Use of immunosuppressants such as methotrexate or azathioprine will require regular monitoring of CBC and LFTs.

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