Reviewed By: Pramod Kerkar, MD, FFARCSI

What Is Secondary Spontaneous Pneumothorax?

Secondary spontaneous pneumothorax (SSP) is defined as spontaneous occurring of air in the pleural space in patients with underlying lung disease. As the definition says SSP is secondary to an underlying lung disease. Secondary spontaneous pneumothorax is more common in elderly people and the peak incidence is seen in people age 60-65 years. Reason for this is lung disease is seen more with advanced age. The age-adjusted rate of SSP is 6.3 cases per 100,000 persons per year for men and 2.0 cases per 100,000 persons per year for women. The male-to-female ratio of age-adjusted rates is 3.2:1.

What Is Secondary Spontaneous Pneumothorax?

Diseases associated with secondary spontaneous pneumothorax

Airway Diseases

Chronic Obstructive Pulmonary Disease (COPD)/Emphysema: This is the most common cause of Secondary spontaneous pneumothorax, especially when emphysema is present these patients are at an increased risk of developing SSP. Increased pulmonary pressure due to coughing with a bronchial plug of mucus or phlegm bronchial plug may give rise to Secondary spontaneous pneumothorax.

Cystic Fibrosis: In cystic fibrosis, up to 18.9% of patients have been reported to develop spontaneous pneumothoraces, and they have a high incidence of recurrence on the same side after conservative management (50%) or intercostal drainage (55.2%). The risk of Secondary spontaneous pneumothorax in these patients increases with Burkholderia cepacia or Pseudomonas infections and allergic bronchopulmonary aspergillosis (ABPA).

  • Severe asthma
  • Infectious Lung Diseases

Pneumocystis Jiroveci Pneumonia: Pneumocystis jiroveci pneumonia (PJP) (previously known as Pneumocystis carinii pneumonia) was a common cause of Secondary spontaneous pneumothorax in patients with AIDS during the last decade. About 77% of AIDS patients with spontaneous pneumothorax had thin-walled cavities, cysts, and pneumothorax from PJP infection. With the advent of highly active antiretroviral therapy (HAART) and widespread use of trimethoprim-sulfamethoxazole (TMP-SMZ) prophylaxis, the incidence of PJP and associated SSP has significantly reduced.

  • Tuberculosis
  • Necrotizing pneumonia
  • Interstitial Lung Diseases
  • Idiopathic pulmonary fibrosis
  • Sarcoidosis
  • Histiocytosis X
  • Lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) may present with spontaneous pneumothorax. This disease is characterized by thin-walled cysts in women of childbearing age.

Interstitial lung diseases are associated with connective-tissue diseases. Apical fibrosis is present in patients with ankylosing spondylitis. Incidence of spontaneous pneumothorax in patients with ankylosing spondylitis is about 0.29%, it increases 45-fold (to 13%) when apical fibrotic disease is present.

Malignant Diseases

Many different types of cancers are known to present with a pneumothorax, especially sarcomas, but genitourinary cancers and primary lung cancer can also be present as pneumothorax. Therefore, a pneumothorax in a patient with cancer should prompt a look for metastatic disease. Chemotherapeutic agents, sometimes, can also induce secondary spontaneous pneumothorax.

  • Lung cancer
  • Sarcoma
  • Symptoms of Secondary Spontaneous Pneumothorax
  • Mediastinal and tracheal shift to the contralateral side – occurs when SSP progress into tension pneumothorax
  • Hyperressonance on percussion – rare finding
  • Distant or absent breath sounds in the affected lung
  • Crepitations, ronchi (wheezing) can be present according to the disease
  • The symptoms and signs depend on the severity of the pneumothorax.

Secondary spontaneous pneumothorax most of the time presents as a potentially life threatening disease, requiring immediate medical action and SSP patients can progress into tension pneumothorax.

Summary

Secondary spontaneous pneumothorax (SSP) is defined as spontaneous occurring of air in the pleural space in patients with underlying lung disease. SSP is more common in elderly people and the peak incidence is seen in people age 60-65 years. The disease associated with SSP are COPD/emphysema, cystic fibrosis, severe asthma, Pneumocystis jiroveci pneumonia, tuberculosis, necrotising pneumonia, idiopathic pulmonary disease, connective tissue disorders and malignant disease. They present with shortness of breath, chest pain, and cyanosis. On examination they can be in respiratory failure, tachypnea, hypoxemia, hyercapnia, mediastinal/tracheal shift, distant or absent breath sound and additional sound like crepitations, ronchi.

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Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: October 15, 2018

This article does not provide medical advice. See disclaimer

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