Treatment and prognosis

Important considerations for pulmonary LCH (pLCH)

The natural history is widely variable and mostly unpredictable in the individual patient. About 40 to 50% of patients experience a favorable outcome and partial or complete clearance of the radiological abnormalities occurs with or without therapy.

Serial lung function tests are essential for following patients with PLCH. In a recent retrospective multicenter study, lung function (mainly DLCO and FEV1) deteriorated in approximately 60% of the patients [Tazi et al 2012]. Airway obstruction was the major functional pattern observed. FEV1% of predicted at diagnosis was associated with the incidence of airway obstruction. A subgroup of patients experienced a dramatic decline in FEV1 within two years after diagnosis.

An isolated decline of DLCO in symptomatic patients should prompt a search for pulmonary hypertension by echocardiography and in case of increased systolic pulmonary arterial pressure should be confirmed by right heart catheterization [Le Pavec et al 2012].

Based on the epidemiologically data smoking cessation is generally demanded. Patients with a stable disease despite ongoing smoking should be told about all other known medical reasons for termination and an especial supportive program may be needed. In particular, these patients may develop COPD and are at increased risk of lung cancer. [Tazi 2006; Vassallo et al 2002]

There are no study-based data supporting cortisone therapy for pulmonary LCH. Any possible therapeutic benefit for symptomatic patients should, therefore, be carefully weighed against the potential undesired effects of this form of treatment, because spontaneous remissions do occur. If smoking cessation failed and treatment is required systemic steroid therapy (usually 1mg/kg/day for one month, followed by tapering dosages over months) may be given in patients with the nodular form of pulmonary LCH [Tazi 2006; Vassallo et al 2002].

Lower respiratory tract infection is a common cause of deterioration of PLCH and should lead to prompt treatment. Annual vaccination against influenza as well as anti-pneumococcal is recommended for patients with impaired lung function.

Progressive PLCH despite steroid therapy may be treated with 2-CDA. [Lazor et al 2009; Lorillon et al 2012]. A randomized controlled trial evaluating the effectiveness and tolerance of 2-CDA in this subgroup of patients is ongoing (see table 8).

Pneumothorax and lung transplantation

Pneumothorax requires drainage and pleurodesis should be considered in case of recurrence [Mendez et al 2004].

Lung transplantation (LT) may represent a therapeutic option in case of advanced PLCH (severe respiratory failure or major pulmonary hypertension). Recurrence of LCH after transplatation occurs in 20% without impact on the survival rate [Dauriat et al 2006].

Therapeutic recommendations in pulmonary LCH (pLCH)

Grades of recommendations for therapy in pLCH are listed in table 8.

Table 8: Therapeutic recommendations in pLCH
Recommendation
Grade *
  • First step is smoking secession in all patients
C2
  • Watchful waiting in asymptomatic or minor symptomatic patients
C2
  • Systemic steroid therapy in symptomatic patients
C2
  • Chemotherapy (e.g. 2-CDA) in progressive disease
C2
  • Consider lung transplantation in case of severe respiratory failure or major pulmonary hypertension
C2

* Grades of recommendation see here.