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Goodpasture's syndrome

  1. Definition. Goodpasture's syndrome is a progressive autoimmune disease of the lungs and kidneys that produces intra-alveolar hemorrhage and glomerulonephritis. The disease is rare, occurs at all ages, and is predominant in males.
  2. Pathogenesis and pathology
    1. It is caused by an anti glomerular basement membrane (anti-GBM) antibody, usually IgG, which reacts with glomerular and alveolar basement membranes.
    2. Linear deposition of the antibody, characteristic of a type II hypersensitivity reaction, occurs along the basement membrane of glomeruli, alveoli, and capillaries.
    3. The pathologic result in the lung is diffuse capillary leakage and intra-alveolar hemorrhage but little or no inflammation.
    4. The renal lesion is a proliferative glomerulonephritis that progresses to renal failure.
    5. Clinical features. hemoptysis and dyspnea renal failure.
  3. Diagnosis
    1. Bilateral alveolar infiltrates on chest radiograph, hypoxemia, and a restrictive pattern on pulmonary function testing are characteristic.
    2. The diagnosis is confirmed by demonstration of anti-GBM antibody in the serum or in a biopsy specimen from the kidney or lung.
  4. Prognosis and therapy
    1. Untreated Goodpasture's syndrome is rapidly fatal as a result of renal failure or asphyxia from pulmonary hemorrhage.
    2. Currently, the combination of plasmapheresis to remove circulating anti-GBM antibodies and immunosuppressive therapy with corticosteroids and cyclophosphamide to give the best results.
    3. Bilateral nephrectomy with hemodialysis or kidney transplantation has been used to control end-stage renal disease.

Wegener's granulomatosis

  1. Definition. This disease is the prototype of a group of rare disorders characterized by granulomatous inflammation and necrosis of the lung and other organs. Individuals of all ages may be affected; males are affected more commonly than females.
    1. Disease is a systemic vasculitis with a predilection for the respiratory tract and kidney. Other commonly involved sites are the skin, joints, and peripheral nerves. Involvement of the eyes, heart, and CNS also can occur.
  2. Pathogenesis and pathology
    1. The pathologic lesion is an angiitis of small vessels with characteristic tissue necrosis surrounded by mononuclear inflammatory cells, forming noncaseating granulomas.
      1. In the lung, this process commonly results in excavation and destruction of the lung parenchyma, causing hemoptysis and pulmonary insufficiency.
      2. The renal lesion is a focal glomerulonephritis that can progress to renal failure.
      3. Diagnosis. Wegener's granulomatosis is identified by the classic clinical triad of upper and lower respiratory involvement and glomerulonephritis, supported by a positive anti-neutrophilic cytoplasmic antibody (ANCA) test and biopsy of the involved tissue.
  3. Prognosis and therapy
    1. The untreated disease is fatal in most patients within 1 month to several years.
    2. Cyclophosphamide (drug of choice) alone or with corticosteroids produces rapid reversal of the disease, if introduced early in the course.
    3. Methotrexate, rituximab, etanercept and cotrimoxazole are other drugs. (Ref. Hari. 18th ed., 2792)

Histiocytosis X (eosinophilic granuloma of the lung)

  1. Definition. Histiocytosis X is a generic term for a group of systemic disorders characterized by various degrees of fibrosis with focal infiltrations of tissue by nonmalignant histiocytes and eosinophils. The disease can be localized to one area (e.g., bone or lung) or it can be widely disseminated. Eosinophilic granuloma (of bone or lung) is localized; Letterer-Siwe disease and Hand-Schüller-Christian syndrome are widespread.
  2. Incidence. The disease is rare, affects men more commonly than women, and affects children and young adults more commonly than other age-groups.
  3. Pathology. Proliferating histiocytes show cytoplasmic inclusions, the so-called X bodies. Pulmonary histiocytosis X produces bilateral, reticulonodular infiltrates, with a predilection for the upper lobes and typical progression to cyst formation, fibrosis, and honeycombing.
  4. Clinical features and diagnosis
    1. Findings may include cough, chest pain, dyspnea, fever, spontaneous pneumothorax, and a appearance on chest radiography. Lytic bone disease may be present. A triad of diabetes insipidus, exophthalmos, and bone lesions is seen occasionally.
    2. Pulmonary function testing indicates restriction and impaired gas exchange. In advanced cases, severe obstruction may dominate.
    3. Definitive diagnosis requires biopsy of involved tissue or electron microscopic demonstration of X bodies in BAL fluid.
  5. Therapy and prognosis. Corticosteroids are given for pulmonary manifestations. Surgery or radiation therapy is used for localized bone disease. The prognosis is variable some cases result in death, but spontaneous remissions are common.

Alveolar proteinosis

  1. Definition. Alveolar proteinosis is a rare disease characterized by massive accumulations of a phospholipid- and protein-rich substance in alveoli. The interstitium usually is not involved, and there is no underlying disease or other organ involvement. The disorder is more common in men than in women occur in all ages.
  2. Pathology. The substance in the alveoli is closely related to pulmonary surfactant and accumulates as a result of impaired clearance. The impaired clearance may be due to deficiency or defect of granulocyte macrophage colony-stimulating factor (GM-CSF). Macrophages engorged with the substance also are present, but other inflammatory cells are lacking
  3. Clinical features
    1. Findings. Dyspnea, nonproductive cough, pulmonary rales, and cyanosis are common.
    2. Clinical course and prognosis
      1. Patients are predisposed to lung infection, including nocardiosis and fungal infections, possibly because of a functional impairment of alveolar macrophages.
      2. The disease may progress to respiratory insufficiency and death, but spontaneous resolution is just as common. Pulmonary fibrosis is a late complication.
  4. Diagnosis. Pulmonary function testing shows a restrictive ventilatory pattern and hypoxia. Chest radiograph shows an alveolar infiltrate, usually in a bilateral perihilar butterfly distribution similar to the pattern seen in pulmonary edema. Lung biopsy or BAL is necessary to demonstrate the periodic acid Schiff (PAS)“positive material in the alveoli.
  5. Therapy.
    1. Patients with minimal symptoms require no therapy.
    2. For dyspneic patients, whole lung lavage is effective and reverses the physiologic abnormality.
    3. Corticosteroids are contraindicated because they increase the risk of infection.

Bronchiolitis obliterans-organizing pneumonia (BOOP)

  1. Definition and pathology. BOOP, also called cryptogenic organizing pneumonia (COP), is a clinicopathologic syndrome involving granulation tissue within small airways and alveolar ducts. It is associated with chronic inflammation in the surrounding alveoli.
  2. Etiology. Viral infection, toxic inhalation, rheumatoid arthritis, and other collagen vascular diseases, as well as drugs, are some of the potential causes of BOOP.
  3. Clinical features
    1. BOOP affects both men and women. The mean age at presentation is 58 years; patients range in age from 21 to 80 years.
    2. The disease usually presents as a subacute flu-like illness with coughing, fever, malaise, fatigue, and weight loss. Inspiratory crackles are frequently present.
  4. Diagnosis
    1. Chest radiography shows bilateral, peripheral, wedged-shaped alveolar opacities.
    2. Pulmonary function testing shows a restrictive defect.
    3. Lung biopsy is the definitive way to make a diagnosis.
  5. Therapy. In two-thirds of patients, corticosteroid therapy results in rapid and complete recovery.

Pancoast’s tumor

Pancoast syndrome is characterized by a malignant neoplasm of the superior sulcus of the lung with destructive lesions of the thoracic inlet and involvement of the brachial plexus and cervical sympathetic nerves (stellate ganglion).
This is accompanied by the following:
  1. TRIAD
    1. Severe pain in the shoulder region radiating toward the axilla and scapula along the ulnar aspect of the muscles of the hand
    2. Atrophy of hand and arm muscles
    3. Horner syndrome (ptosis, miosis, hemianhidrosis, enophthalmos)
  2. Compression of the blood vessels with edema
  3. Most Pancoast tumors are squamous cell carcinomas (SCCs) only 3-5% are small cell carcinomas. 
  4. Adenocarcinoma is sometimes found in this location and can even be metastatic. 
  5. Involvement of the phrenic or recurrent laryngeal nerve or superior vena cava obstruction is not representative of the classic Pancoast tumor.
  6. Investigation - MRI

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