What are risk factors for secondary spontaneous pneumothorax (SSP)?

Updated: Apr 28, 2020
  • Author: Brian J Daley, MD, MBA, FACS, FCCP, CNSC; Chief Editor: Mary C Mancini, MD, PhD, MMM  more...
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Answer

SSPs occur in the presence of lung disease, primarily in the presence of COPD. Other diseases that may be present when SSPs occur include tuberculosis, sarcoidosis, cystic fibrosis, malignancy, and idiopathic pulmonary fibrosis.

Pneumocystis jiroveci pneumonia (previously known as Pneumocystis carinii pneumonia [PCP]) was a common cause of SSP in patients with AIDS during the last decade. In fact, 77% of AIDS patients with spontaneous pneumothorax had thin-walled cavities, cysts, and pneumothorax from PCP infection. [11] With the advent of highly active antiretroviral therapy (HAART) and widespread use of trimethoprim-sulfamethoxazole (TMP-SMZ) prophylaxis, the incidence of PCP and associated SSP has significantly declined.

PCP in other immunocompromised patients is seen only when TMP-SMZ prophylaxis is withdrawn prematurely. For practical purposes, if the immunocompromised patient has been taking TMP-SMZ prophylaxis reliably, PCP is reasonably excluded from the differential diagnosis and should not be a causative factor for SSP.

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 SSP in these patients increases with Burkholderia cepacia or Pseudomonas infections and allergic bronchopulmonary aspergillosis (ABPA). [12] Pleurodesis increases the risk of bleeding associated with lung transplantation but is not an absolute contraindication.

Many different types of malignancies are known to present with a pneumothorax, especially sarcomas, but also genitourinary cancers and primary lung cancer; thus, pneumothorax in a patient with malignancy should prompt a look for metastatic disease. Chemotherapeutic agents, at times, can also induce SSP. [13]

Interstitial lung diseases are associated with connective-tissue diseases. Ankylosing spondylitis may be noted when apical fibrosis is present; in fact, the typically low incidence of spontaneous pneumothorax in patients with ankylosing spondylitis (0.29%) increases 45-fold (to 13%) when apical fibrotic disease exists. [14]

Lymphangioleiomyomatosis (LAM) may present with spontaneous pneumothorax. This disease is characterized by thin-walled cysts in women of childbearing age. Respiratory failure may lead to a need for lung transplantation, and previous pleurodesis is no longer an absolute contraindication for lung transplantation.

Thoracic endometriosis is a rare cause of recurrent pneumothorax (catamenial pneumothorax) in women that is thought to arise from endometriosis reaching the chest wall across the diaphragm (ie, its etiology may be primarily related to associated diaphragmatic defects). In a case series of 229 patients, catamenial pneumothorax caused by thoracic endometriosis was localized to the visceral pleura in 52% of patients and to the diaphragm in 39% of patients. [15] Before recurrence, this condition may be initially diagnosed as primary spontaneous pneumothorax.


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