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Lymphocytic Interstitial Pneumonia

LIP

Lymphocytic interstitial pneumonia is a rare, slowly progressive pulmonary disorder associated with autoimmune diseases, dysgammaglobulinemia, human immunodeficiency virus infection and Epstein-Barr virus infection or it could be idiopathic. It is characterized by inflammatory alveolar infiltration leading to clinical manifestations. Imaging studies and lung biopsy are necessary to confirm the diagnosis.


Presentation

The etiology of lymphocytic interstitial pneumonia (LIP) is unknown. It is characterized by infiltration of the pulmonary alveoli by inflammatory lymphocytes, plasma cells and histiocytes [1]. LIP is often associated with autoimmune conditions like Sjögren's syndrome, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Hashimoto thyroiditis as well as immunodeficient diseases like human immunodeficiency virus infection (HIV), combined variable immunodeficiency, agammaglobulinemia, and Epstein-Barr virus (EBV) infection [2] [3] [4].

Although LIP has been reported in patients of all age groups, a majority of the patients are middle-aged and the incidence is higher amongst females [5]. If LIP occurs in a pediatric patient, then it should raise suspicion of underlying acquired immunodeficiency syndrome (AIDS).

Patients with LIP often do not present any signs of the disease and are diagnosed incidentally based on radiological findings [6]. Symptoms when present can be nonspecific such as a long-standing cough becoming worse with time, dyspnea, chest pain, rarely hemoptysis [6], anorexia, fever, night sweats and joint pain. Clinical manifestations of associated diseases like parotid enlargement, xerophthalmia and xerostomia [7] can also be present. The features in children are slightly different and can include clubbing, dyspnea, lymphadenopathy, parotid enlargement, and hepatosplenomegaly.

As the disease progresses, patients develop bronchitis, bronchiectasis and eventually, pulmonary fibrosis. The incidence of respiratory failure with cyanosis is higher amongst children with LIP.

Cough
  • She presented with dyspnea, cough, weight loss, and bibasilar pulmonary infiltrates. Lung biopsy demonstrated lymphocytic interstitial pneumonia and corticosteroid therapy resulted in clinical and radiological improvement.[ncbi.nlm.nih.gov]
  • It is characterized clinically by the presence of cough and dyspnea, diffuse pulmonary infiltrates on chest x-ray, restrictive pulmonary dysfunction, and hypoxemia.[ncbi.nlm.nih.gov]
  • A 59-year-old woman was admitted to our hospital complaining of a productive cough, dyspnea on effort, and low-grade fever. Although chest X-rays showed no marked abnormalities, her level of serum KL-6 was extremely high.[ncbi.nlm.nih.gov]
  • All patients presented with progressive dyspnea, nonproductive cough, fever, anorexia, weight loss, and arterial hypoxemia. Chest roentgenograms exhibited bilateral diffuse reticular-nodular densities.[ncbi.nlm.nih.gov]
  • A five-year-old male, infected by human immunodeficiency virus (HIV), was admitted at the division of infectious diseases of this hospital with cough, tachydyspnea, fever, and breathing difficulty.[ncbi.nlm.nih.gov]
Dyspnea
  • Exertional dyspnea, x-ray infiltrates, and pulmonary physiologic abnormalities decreased or increased during 3 1/2 years of follow-up in parallel with the administered dose of prednisone.[ncbi.nlm.nih.gov]
  • The features in children are slightly different and can include clubbing, dyspnea, lymphadenopathy, parotid enlargement, and hepatosplenomegaly.[symptoma.com]
  • A 62-year-old Japanese female was admitted due to dyspnea. She showed a marked increase in CA19-9 (maximum, 192,000 U/ml) and monoclonal IgM-kappa type rheumatoid factor (RF) activity. The patient died of respiratory failure 3 months later.[ncbi.nlm.nih.gov]
  • She presented with dyspnea, cough, weight loss, and bibasilar pulmonary infiltrates. Lung biopsy demonstrated lymphocytic interstitial pneumonia and corticosteroid therapy resulted in clinical and radiological improvement.[ncbi.nlm.nih.gov]
  • It is characterized clinically by the presence of cough and dyspnea, diffuse pulmonary infiltrates on chest x-ray, restrictive pulmonary dysfunction, and hypoxemia.[ncbi.nlm.nih.gov]
Dry Cough
  • Breathlessness during exercise is one of the initial symptoms of these diseases, while a dry cough may also be present. People with different types of ILD may have the same kind of symptoms but their symptoms may vary in severity.[books.google.com]
  • Here we present a case of LIP in an immunocompetent adult male presented with history of persistent dry cough and breathlessness on exertion, confirmed on HRCT chest and histopathologically, treated successfully with steroids.[lungindia.com]
  • AIDS); especially in children Monoclonal or polyclonal gammopathy Common variable immunodeficiency Idiopathic LIP accounts for 20% of cases ( Eur Respir J 2006;28:364 ) Clinical features Very slowly progressive respiratory symptoms Dyspnea on exertion Dry[pathologyoutlines.com]
  • The symptoms will vary from person to person, but the most common symptoms of an ILD are: Shortness of breath, especially with exertion or exercise Dry cough that does not produce phlegm Labored breathing Although rapid worsening can happen, ILD's symptoms[uchospitals.edu]
  • A dry cough is another symptom. Symptoms often get worse over time. See your doctor if you have trouble breathing. After a diagnosis, you can start treatments to manage the inflammation and scarring.[healthline.com]
Rales
  • During the physical examination, chest auscultation can reveal rales and clubbing.[symptoma.com]
  • Pleural effusion: uncommon (suggests presence of complicating lymphoma) Hypo or hypergammaglobulinemia: may be seen Serum autoantibodies or immune complexes: may be positive Clinical Clubbing (common) Dyspnea: Cough: Pleuritic Chest Pain: Bibasilar Rales[mdnxs.com]
  • […] loss of volume is characteristic of either UIP or scleroderma Clinically Symptoms include SOB, non-productive cough and fatigue Clubbing of the fingers is very common (85%) Cyanosis and pulmonary hypertension usually occur late in the disease “Velcro” rales[learningradiology.com]
  • Bibasilar rales are a consistent finding on lung auscultation (1,3,4,16); wheezes and decreased breath sounds may also be present (4). However, in many instances, the physical findings are minimal (1) or the physical examination is normal (1,12).[path.upmc.edu]
  • […] examination findings in children may include the following: Generalized lymphadenopathy Hepatosplenomegaly Parotid enlargement Clubbing Wheezing (occasional) Physical examination findings in adults may include the following: Generalized lymphadenopathy Rales[emedicine.medscape.com]
Pulmonary Disorder
  • Lymphocytic interstitial pneumonia is a rare, slowly progressive pulmonary disorder associated with autoimmune diseases, dysgammaglobulinemia, human immunodeficiency virus infection and Epstein-Barr virus infection or it could be idiopathic.[symptoma.com]
  • Previous Contents Next Lymphocytic interstitial pneumonitis (LIP) LIP is a non-infectious pulmonary disorder caused by white cell infiltration into alveolae.[books.mcai.org.uk]
  • Reactive pulmonary disorders. Histopathology 1995;26:405-412. Teirstein AS, Rosen MJ. Lymphocytic interstitial pneumonia. Clin Chest Med 1988;9:467-471. Yousem SA, Colby TV, Carrington CB. Follicular bronchitis/bronchiolitis.[path.upmc.edu]
  • Immunosuppressive and cytotoxic pharmacology for pulmonary disorders. Am J Respir Crit Care Med 1997 ; 155 : 395 –420. 26. Bates CA, Ellison MC, Lynch DA, Cool CD, Brown KK, Routes JM.[erj.ersjournals.com]
Vietnamese
  • We describe a case of pSS in a 14-year-old Vietnamese-Canadian girl who presented with pulmonary symptoms, radiologic changes, and biopsy confirmation of lymphocytic interstitial pneumonia.[ncbi.nlm.nih.gov]
Cyanosis
  • The incidence of respiratory failure with cyanosis is higher amongst children with LIP. The diagnosis of LIP is often delayed due to the nonspecific symptoms. A clinical suspicion is required to detect the condition.[symptoma.com]
  • ) Dyspnea: Cough: Pleuritic Chest Pain: Bibasilar Rales: Pulmonary HTN: late Other System Involvement: a) Lymphadenopathy: b) Keratoconjunctivitis sicca: c) Salivary Gland Enlargement: d) Hepatosplenomegaly: e) Fever: f) Weight Loss: g) Clubbing: h) Cyanosis[mdnxs.com]
  • Becomes coarser as disease progresses and ends with honeycomb lung Progressive loss of volume is characteristic of either UIP or scleroderma Clinically Symptoms include SOB, non-productive cough and fatigue Clubbing of the fingers is very common (85%) Cyanosis[learningradiology.com]
  • If the disease progresses to the end-stage respiratory failure cyanosis and clubbing may develop. Hypertrophy of the salivary glands observed in 20% of patients 11 .[radiopaedia.org]
  • His general physical examination was normal, with pulse rate of 88/minute, blood pressure 110/68 mmHg, respiratory rate 22/minute, J.V.P. not raised, cyanosis, lymphadenopathy, clubbing, were not present.[lungindia.com]
Parotid Swelling
  • The child is often asymptomatic in the early stages, but may later have a mild persistent cough, with or without difficulty in breathing, bilateral parotid swelling, persistent generalised lymphadenopathy, poor growth, hepatomegaly and other signs of[books.mcai.org.uk]

Workup

The diagnosis of LIP is often delayed due to the nonspecific symptoms. A clinical suspicion is required to detect the condition. Patients may provide a history of progressively worsening symptoms. During the physical examination, chest auscultation can reveal rales and clubbing.

Routine laboratory tests will show elevated levels of inflammatory markers in the presence of autoimmune disease and polyclonal agammaglobulinemia or dysgammaglobulinemia on serum protein electrophoresis. Tests for HIV, EBV, RA factor, SLE, Sjögren's and Hashimoto thyroiditis should be performed to detect the disorders associated with LIP. Lactate dehydrogenase (LDH) levels are obtained in pediatric patients to differentiate LIP from pneumocystis carinii pneumonia.

Restrictive pattern and diminished diffusion capacity are seen on pulmonary function testing [8].

Plain X-ray chest may show bilateral basal opacities and cysts. Computed tomography (CT) scan is obtained to determine the extent of lung involvement and for subsequent follow-up of the disease. Findings include cysts, opacified nodules, bronchiectasis, bullae with air bronchogram and pulmonary fibrosis in the late stages of LIP [9] [10].

Bronchoalveolar lavage is performed to exclude infection and is likely to reveal lymphocytic infiltrate [8].

However, confirmation of diagnosis requires histological examination of a lung biopsy specimen [6]. The typical features of LIP are widening of the alveolar septae with extensive interstitial lymphocytic, plasma cell and histiocytic infiltration. Immunohistochemical staining and flow cytometry are required to differentiate between LIP and pulmonary lymphoma. Polyclonal infiltrate (i.e. both T and B cells) is seen in LIP, while monoclonal infiltrate with destruction/distortion of the airways and vessels and pleural involvement are a feature of lymphomas [2].

Pulmonary Infiltrate
  • After a diagnostic surgical lung biopsy, his hypoxemia and pulmonary infiltrates exacerbated rapidly over a few days, although his condition had not progressed during the previous year.[ncbi.nlm.nih.gov]
  • The widespread interstitial pulmonary infiltration in two patients, showing the clinical, radiological and histological features of lymphocytic interstitial pneumonia (LIP), has been characterized by histological and immunological criteria as malignant[ncbi.nlm.nih.gov]
  • She presented with dyspnea, cough, weight loss, and bibasilar pulmonary infiltrates. Lung biopsy demonstrated lymphocytic interstitial pneumonia and corticosteroid therapy resulted in clinical and radiological improvement.[ncbi.nlm.nih.gov]
  • It is characterized clinically by the presence of cough and dyspnea, diffuse pulmonary infiltrates on chest x-ray, restrictive pulmonary dysfunction, and hypoxemia.[ncbi.nlm.nih.gov]
  • Overview Overview Lymphocytic interstitial pneumonia (LIP) is a syndrome of fever, cough, and dyspnea, with bibasilar pulmonary infiltrates consisting of dense interstitial accumulations of lymphocytes and plasma cells.[emedicine.medscape.com]
Reticulonodular Pattern
  • Eight children of 12 had a reticulonodular pattern on chest radiographs. Two children had normal chest films and two children showed peribronchiolar thickening.[ncbi.nlm.nih.gov]
  • pattern (most common) 2) Mixed alveolar-interstitial infiltrates: due to coalescence 3) Hilar/ mediastinal nodes: in some cases 4) Honeycombing: small cysts (seen best in lower fields) seen late/ radiographic honeycombing correlates well with pathologic[mdnxs.com]
Chlamydia Trachomatis
  • Epstein-Barr virus and Chlamydia trachomatis are potential etiologic agents, but a specific cause remains to be identified. This disorder has been described with a higher frequency in pediatric AIDS.[ncbi.nlm.nih.gov]

Treatment

  • However, responses to different treatments are widely varied, and no single first line treatment represents the default treatment for lymphocytic interstitial pneumonia. [5] References [ edit ] The Pathology of Idiopathic Interstitial Pneumonias Carol[en.wikipedia.org]
  • Because of severe general condition of the patient, immunosuppressive treatment was started immediately, without further invasive diagnostics including lung biopsy, which is required for a definitive diagnosis.[ncbi.nlm.nih.gov]
  • Treatment includes oxygen and bronchodilators as needed. The role of zidovudine and of steroids in the management of LIP remains to be determined.[ncbi.nlm.nih.gov]
  • (IgG normal range: 6–16 g/l; IgM normal range: 0.4–2.4 g/l; IgA normal range: 0.8–4.0 g/l). ( E ) ESR during course of treatment (normal range: 1–12 mm/h). ( F ) CRP during course of treatment (normal range: D – F ) Boxes relate to treatment periods[academic.oup.com]
  • We believe that, in selected cases of LIP and immunodeficiency, IVIG given monthly should be considered as the only treatment without adding steroids.[ncbi.nlm.nih.gov]

Prognosis

  • Although the clinical presentation may be identical to the more common opportunistic infections, the treatment differs, and the prognosis may be better.[ncbi.nlm.nih.gov]
  • Low-Grade Pulmonary or Systemic Lymphoma: may occur in some cases 2) Infection (especially in cases with associated dysproteinemia): Treatment Steroids: may markedly improve disease (cited in few case reports) Some cases progress to death despite therapy Prognosis[mdnxs.com]
  • Prognosis and Treatment Approximately 33% to 50% of patients with LIP die within 3 to 5 years. However, prognosis depends somewhat on the associated underlying disease condition. In some patients the disease stabilizes or resolves.[humpath.com]
  • Treatment and prognosis With smoking cessation and corticosteroid therapy, the prognosis is good. The overall prognosis of has been described as being significantly better than that of fibrotic NSIP 11 .[radiopaedia.org]
  • Vrations in histilogical patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology 2004;44:585-96. 8. Lee HK, Kim DS, Yoo B, Seo JB, Rho JY, Colby TV, et al .[lungindia.com]

Etiology

  • Epstein-Barr virus and Chlamydia trachomatis are potential etiologic agents, but a specific cause remains to be identified. This disorder has been described with a higher frequency in pediatric AIDS.[ncbi.nlm.nih.gov]
  • Evidence of an autoimmune etiology includes its frequent association with Sjögren syndrome (25% of cases of LIP) and other disorders (eg, SLE , RA , Hashimoto thyroiditis —14% of cases).[msdmanuals.com]
  • The etiology of lymphocytic interstitial pneumonia (LIP) is unknown. It is characterized by infiltration of the pulmonary alveoli by inflammatory lymphocytes, plasma cells and histiocytes.[symptoma.com]
  • The etiology of LIP remains unclear. The vast spectrum of disorders associated with this condition further complicates investigation. In 1973, Liebow and Carrington suggested multiple possible etiologies, including a viral agent (1).[path.upmc.edu]
  • Epidemiology Sex: more common in females than males Age: usually presents in 30’s-50’s (may be seen in children, particularly those with hypogammaglobulin-emia or AIDS) Etiology Dysproteinemia Hypogammaglobulinemia Monoclonal or Polyclonal Gammopathy[mdnxs.com]

Epidemiology

  • Epidemiology Sex: more common in females than males Age: usually presents in 30’s-50’s (may be seen in children, particularly those with hypogammaglobulin-emia or AIDS) Etiology Dysproteinemia Hypogammaglobulinemia Monoclonal or Polyclonal Gammopathy[mdnxs.com]
  • […] immunodeficiency virus (HIV) infection On histology, diffuse infiltration of polyclonal lymphocytes with scant interstitial fibrosis is characteristic Terminology Also called lymphocytic interstitial pneumonia ICD-10 coding J84.2 : lymphoid interstitial pneumonia Epidemiology[pathologyoutlines.com]
  • Oral Oncol 34:284–291. 7- Ho T, Wei Q, Sturgis EM (2007) Epidemiology of carcinogen metabolism genes and risk of squamous cell carcinoma of the head and neck.[otorrinocontiplanes.com]
  • Summary Epidemiology The incidence of HP is unknown. A population-based study estimated the annual incidence of interstitial lung diseases as 1/3300 and HP accounted for less than 2% of these cases.[orpha.net]
  • Epidemiology Lymphocytic interstitial pneumonia (LIP) is an uncommon disease. In the United States, however, it is found in 22-75% of pediatric patients with HIV who have pulmonary disease.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • […] coding J84.2 : lymphoid interstitial pneumonia Epidemiology Rare Typical onset at ages 40 - 70 years old but can occur at any age ( Chest 2002;122:2150 ) More common in women No association with smoking history Sites Bilateral lower lobes of the lung Pathophysiology[pathologyoutlines.com]
  • For other discussions on pneumonia, see the following: Pathophysiology and Etiology HIV-related lymphocytic interstitial pneumonia (LIP) may be part of a continuum of lymphocytic infiltrative disorders, such as pulmonary lymphoid hyperplasia in children[emedicine.medscape.com]
  • Liebow AA, Carrington CB: The interstitial pneumonias, in Simon M, Potchen EJ, Le May M (eds): Frontiers of Pulmonary Radiology: Pathophysiologic, Roentgenographies and Radioisotopic Considerations . New York, Grune & Stratton, 1969, pp 102–141.[link.springer.com]
  • Immunodiagnostic and pathophysiologic aspects of antineutrophil cytoplasmic antibodies in vasculitis . Curr Opin Rheumatol 1995; 7 : 11–19. 71. Edgar JD , McMillan SA , Bruce IN , Conlan SK . An audit of ANCA in routine clinical practice .[nature.com]

Prevention

  • Ziegler JL, Abram DI: The AIDS related complex, in DeVita VT Jr, Hellman S, Rosenberg SA (eds): AIDS: Etiology, Diagnosis, Treatment, and Prevention . New York, JB Lippincott, 1985, pp 223–234. Google Scholar 22.[link.springer.com]
  • Can interstitial lung disease be prevented? Interstitial lung disease can be prevented only to the extent that its individual causes may be prevented.[medicinenet.com]
  • Antifibrotic drugs such as pirfenidone (Esbriet) and nintedanib (Ovef) may prevent further scarring in the lungs. These drugs are both approved by the U.S. Food and Drug Administration for the treatment of idiopathic pulmonary fibrosis.[healthline.com]
  • In children with HIV infection, lymphocytic interstitial pneumonia has been designated an AIDS-defining illness by the US Centers for Disease Control and Prevention. LIP is more common in women when not associated with HIV infection.[emedicine.medscape.com]

References

Article

  1. Kim J-Y, Park S-H, Kim S-K, et al. Lymphocytic interstitial pneumonia in primary Sjogren's syndrome: a case report. Korean J Intern Med. 2011 Mar; 26 (1): 108 -111
  2. Panchabhai TS, Farver C, Highland KB. Lymphocytic interstitial pneumonia. Clin Chest Med. 2016 Sep 3; 37(3): 463-74
  3. Swigris JJ, Berry GJ, Raffin TA, Kuschner WG. Lymphoid interstitial pneumonia: a narrative review. Chest. 2002;122:2150–2164.
  4. Alkhayer M, McCann BG, Harrison BD. Lymphocytic interstitial pneumonitis in association with Sjogren's syndrome. Br J Dis Chest. 1988;82:305–309.
  5. Ferguson EC, Berkowitz EA. Lung CT: Part 2, The interstitial pneumonias-clinical, histologic, and CT manifestations. AJR Am J Roentgenol. 2012;199 (4): W464-76.
  6. Wu W, Zhou J, Di L-G, Chen H. From lymphocytic interstitial pneumonia to MALT lymphoma of lung: a case report with a 5-year diagnostic dilemma. 2015; 8(8): 9698-9702
  7. Couderc LJ, D'Agay MF, Danon F, et al. Sicca complex and infection with human immunodeficiency virus. Arch Intern Med. 1987 May; 147(5):898-901.
  8. Cha S-I, Fessler MB, Cool CD, et al. Lymphoid interstitial pneumonia: clinical features, associations, and prognosis. Eur Respir J. 2006; 28:364-369
  9. Becciolini V, Gudinchet F, Cheseaux JJ, Schnyder P. Lymphocytic interstitial pneumonia in children with AIDS: high-resolution CT findings. Eur Radiol. 2001;11(6):1015-20.
  10. Johkoh T, Ichikado K, Akira M, et al. Lymphocytic interstitial pneumonia: follow-up CT findings in 14 patients. J Thorac Imaging. 2000. Jul; 15(3):162-7.

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Last updated: 2018-06-21 17:45