Sarcoidosis Causes Diagnosis Symptoms and Treatment

Sarcoidosis

It is a chronic, multisystem disorder of unknown cause.

  • chronic multisystem disease of unknown etiology, characterized by noncaseating (hard) granulomas and lymphocytic alveol,
  • Sarcoidosis is a noninfectious, multisystem granulomatous disease of unknown cause, commonly affecting young and middle-aged adults.
  • Almost any other organ may be involved, including liver, spleen, lymph nodes, heart, and CNS.
  • Also can be diagnosed in asymptomatic patients with abnormal CXRs
  • Frequently presents with hilar adenopathy, pulmonary infiltrates, ocular and skin lesions
  • Systems affected: Primarily Pulmonary but also Cardiovascular; GI; Hematologic/Lymphatic; Endocrine; Renal; Neurologic; Dermatologic; Ophthalmologic; Musculoskeletal

Sarcoidosis Diagnosis Characterized by :

  • · T Iymphocytes and phagocytes
  • · Granulomas (non-caseating) in various organs.
  • No definitive test for diagnosis, but diagnosis is suggested by the following:
  • Clinical and radiographic manifestations
  • Exclusion of other diagnoses
  • Histopathologic detection of noncaseating granulomas
Sarcoidosis

Sarcoidosis Causes Diagnosis Symptoms and Treatment

Organs most commonly involved:

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

Course of Disease may be

  • · Acute ,
  • · Sub-acute
  • · Chronic.

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 signs 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

  • · Hepatomegaly
  • · Usually mild features
  • · Jaundice
  • · Portal hypertension.

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 cardiac, 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 drug .

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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