Sarcoidosis Causes Diagnosis Symptoms and Treatment

Sarcoidosis

It is a chronic, multisystem disorder of unknown cause.

Sarcoidosis DiagnosisAlcoholic Cirrhosis Clinical features Physical findings Treatment. Read more ... » Characterized by :

Organs most commonly involved:

  • · Lung
  • · Skin
  • · Eye
  • · Liver

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 bodiesConch-like (Shankh)
  • · Asteroid bodies.

Sarcoidosis Clinical symptoms

  • · Systemic disease
  • · Lungs involved-respiratory symptoms mainly
  • · May be asymptomatic (discovered on chest x­ray)
  • · May present abruptly (suddenly) or slowly in weeks or months
  • · Usually at ages <40 yrs.
  • If signsAlcoholic Cirrhosis Clinical features Physical findings Treatment. Read more ... » indicate Löfgren syndrome, it is not necessary to perform a biopsy because prognosis is good with observation alone, and biopsy would not change management.

Constitutional symptoms are present:

  • · Fever, fatigue, anorexia (loss of appetite), weight loss.

Respiratory symptoms: Cough, 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

  • · Interstitial lung disease
  • · Alveoli, small bronchi, small blood vessels in- volved
  • · There is dyspnoea, dry cough
  • · There are dry rales in lungs
  • · Hemoptysis may be present
  • · Lung collapse can occur due to : – Endobronchial sarcoidosis
  • – Pressure from enlarged lymph nodes
  • · Wheezing may be present.

Necrotizing sarcoidal granulomatosis

  • · Pulmonary artery – granulomatous arteritis
  • · Pleural effusion – usually unilateral
  • · Chronic pleural thickening
  • · Pneumothorax
  • · Hydropneumothorax.

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

  • · Can cause blindness
  • · Can involve uveal tract, iris, ciliary body, choroid, conjunctiva
  • ·Blurred vision, photophobia.

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

  • · Seventh nerve involvement with facial paralysis sudden, transient (for short period)
  • · Optic nerve dysfunction
  • · Papilledema
  • · Hypothalamic and pituitary abnormalities
  • · Chronic meningitis
  • · Granulomas (space occupying lesions)
  • · Seizures
  • · Peripheral neuropathy
  • · Spinal disease.

Musculoskeletal system

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

Heart

  • • LVH
  • .’ Arrhythmias
  • · Conduction defects (CHB)
  • · Sudden death
  • · Papillary muscle dysfunction (PMD)
  • · Pericarditis
  • · CHF
  • · Corpulmonale.

Endocrine and Reproductive system

  • · Diabetes insipidus
  • · Hypopituitarism
  • · Addison’s syndrome,

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 glucocorticoids : 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

  • No treatment may be necessary in asymptomatic individuals, but treatment may be needed for specific indications, such as cardiacMYOCARDITIS Physical Examination Symptoms Clinical Manifestations Diagnosis. Read more ... », CNS, or ocular involvement.
  • ›Systemic therapy is clearly indicated for hypercalcemia, cardiac disease, neurologic disease, and eye disease not responding to topical therapy.
  • ›Treatment of pulmonary and skin manifestations is done on the basis of impairment. The symptoms that necessitate systemic therapy remain controversial.
  • Systemic corticosteroids in the symptomatic individual:
  • ›If no relapse, 15–20 mg/d × 8–12 months
  • ›If stable, taper by 5 mg/wk to 15–20 mg/d over the next 6 weeks
  • ›Relapse is common.
  • ›Usually prednisone initially, 40–60 mg/d × 1st 6 weeks
  • Contraindications: Patients with known problems with corticosteroids
  • In patients with skin or ocular disease, topical steroids may be effective.
  • Precautions: Careful monitoring in patients with diabetes mellitus and/or hypertension
  • Significant possible interactions: Refer to the manufacturer’s profile of each drugAlcoholic Cirrhosis Clinical features Physical findings Treatment. Read more ... » .

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-tumor 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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