of smoking. Symptoms will stabilize in the majority of patients with no need for further treatment. 8 Cessation of smoking can also reduce incidence of associated malig- nancies. Corticosteroid therapy is another option that has demonstrated beneficial effects in stabilization. 3 However, no randomized trials have been conducted to compare the efficacy of corticosteroid treatment with that of smoking cessation alone. Severe PLCH has been treated with che- motherapeutic agents (cladribine, cyclophosphamide, and methotrexate) without significant effect. 7 Pleurodesis may be required in patients with recurrent pneumothoraces. Lung transplantation should also be considered for patients with rapidly deteriorating lung function or disease refractory to medical treatment. Patients with PLCH generally have a good prognosis. 8 However, it has been shown that patients have a decreased average survival rate compared to the general population and have a poorer health-related quality of life than would be expected. 6,7 Spontaneous regression has been reported in up to 25% of patients and stabilization in up to 50% of patients. The remaining 25% of patients follow a variably deteriorating course culminating in diffuse cystic change and lung destruction, sometimes complicated by pulmonary hypertension or respiratory insufficiency leading to death. 2 Relapse may occur, even with lung transplantation, especially if the patient continues to smoke. Long-term follow-up of PLCH patients is necessary because of the risk of relapse, even years after disease resolution. Adult patients with PLCHare at an increased risk for developingmalignant neoplasm of the lung, as well as lymphoma as mentioned earlier. 3 However, this may be due to the high prevalence of cigarette smoking. Patients should be monitored for these complications. REFERENCES 1. Gaensler EA, Carrington CB: Open biopsy for chronic diffuse infiltrative lung disease: clinical, roentgenographic, and physiological correlations in 502 patients. Ann Thorac Surg 1980, 30(5):411-426. 2. ThomeerM, DemedtsM, Vandeurzen K. Registration of interstitial lung diseases by 20 centers of respiratory medicine in Flanders. Acta Clin Belg 2001;56:163-722002;166:1483-90. 3. Tazi A, Soler P, Hance AJ. “Adult Pulmonary Langerhans’ cell histiocytosis.” Thorax . 2000;55:405-416 4. Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH: Clinical outcomes of pulmonary Langerhans’-cell histiocytosis in adults. Semin Respir Crit Care Med . 2002 Apr;23(2):93-101 5. Varkki S, Tergestina M, Bhonsle VS, Moses PD, Mathai J, Korula S. “Isolated Pulmonary Langerhans Cell Histiocytosis” Indian J Pediatr . 2012 Sep 23. [Epub ahead of print] 6. Juvet SC, Hwang D, Downey GP. “Rare lung diseases III: Pulmonary Langerhans’ cell histiocytosis.” Can Respir J . 2010May/ June; 17(3): 55-62 7. Suri HS, Yi ES, Nowakowski GS, Vassallo R. “Pulmonary Langerhans Cell Histiocytosis.” Orphanet Journal of Rare Diseases . 2012; 7(16): 1-13 8. Abbot GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. “From the Archives of the AFIP: Pulmonary Langerhans
Cell Histiocytosis.” Radiographics . 2004 May-Jun;24(3):821-41 9. Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the Archives of AIRP: Pulmonary Langerhans Cell Histiocytosis. Radiographics 2004; 24:821-841. 10. Brauner MW, Grenier P, Moueli MM, Mompoint D, Lenoir S. Pulmonary histiocytosis X: evaluation with high-resolution CT. Radiology 1989; 172:225-258.
Ms. Bauer is a Fourth-Year Medical Student at Tulane University School of Medicine in New Orleans, Louisiana. Dr. Manning is a PGY-5 Radiology Resident at Tulane University Health Sciences Center in New Orleans, Louisiana. Dr. Neitzschman is a Professor of Radiology and the Chairman of the Department of Radiology at Tulane University Health Sciences Center in New Orleans, Louisiana.
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