|Year : 2018 | Volume
| Issue : 4 | Page : 363-365
Idiopathic lymphocytic interstitial pneumonia
Chinnu Sasikumar, Ketaki Utpat, Jyotsna M Joshi
Department of Pulmonary Medicine, TNMC and BYL Nair Hospital, Mumbai, Maharashtra, India
|Date of Web Publication||2-Aug-2018|
Jyotsna M Joshi
Department of Pulmonary Medicine, 2nd Floor, OPD Building, TNMC and BYL Nair Hospital, Al Nair Road, Mumbai Central, Mumbai - 400 008, Maharashtra
Source of Support: None, Conflict of Interest: None
Lymphocytic interstitial pneumonia (LIP) is one of the rare interstitial lung diseases exhibiting a profuse infiltration of lymphoid cells in the interstitium of the lung. It has been classified as one of the rare idiopathic interstitial pneumonias (IIPs) as per the recent American Thoracic Society/European Respiratory Society classification of IIPs. It is an uncommon entity in itself with the idiopathic variety being prodigiously singular. Diagnosis is accomplished by collaboration of clinicoradiological and histopathological attributes. Corticosteroids form the mainstay of therapy with a protean response to therapy. We hereby report a distinctive case of idiopathic LIP picked up inadvertently during a preoperative evaluation.
Keywords: Corticosteroids, idiopathic interstitial pneumonia, lymphoid interstitial pneumonia
|How to cite this article:|
Sasikumar C, Utpat K, Joshi JM. Idiopathic lymphocytic interstitial pneumonia. Med J DY Patil Vidyapeeth 2018;11:363-5
| Introduction|| |
Lymphoid interstitial pneumonia (LIP) is a poorly understood lymphoproliferative disorder with its origin rooted in the hyperplasia of mucosa-associated lymphoid tissue (MALT). LIP is usually found in association with certain infectious diseases such as HIV and Epstein–Barr virus and autoimmune conditions such as Sjögren's syndrome. However, some cases could be diagnosed as idiopathic after an exhaustive evaluation excluding all known causes.
| Case Report|| |
A 52-year-old female nonaddict presented to our outpatient department for the purpose of preoperative assessment. She was to undergo an incisional hernia repair and was referred in view of her chest radiograph (CXR) findings. She was relatively asymptomatic from respiratory point of view and on probing gave a history of only grade 1 dyspnea as per modified medical research council grading. There was no history of exposure to organic or inorganic dust, long-term drug abuse, and photosensitivity, dryness of mouth, or Raynaud's phenomenon. She was a diagnosed case of hypertension since 6 years and was on regular medication for the same. Her general and systemic examination was within normal limits. On investigation, hemoglobin was 12 g/dl, total leukocyte count was 9500 mm with neutrophils 64% and lymphocytes 38%. Other biochemical parameters were within normal limits. CXR [Figure 1] showed bilateral reticulonodular opacities. Rheumatoid arthritis factor, antinuclear antibody, and anti-double-stranded deoxyribonucleic acid titers were within normal limits. HIV panel, hepatitis C virus and hepatitis B antigen, serological tests were nonreactive. Arterial blood gas estimation showed pH 7.34, pCO2 – 40 mmHg, pO2 – 73 mmHg, HCO3 – 2.6.2 mmol/L, and arterial oxygen saturation (SpO2) 96%. High-resolution computed tomography (HRCT) of the chest [Figure 2] showed ill-defined ground-glass opacities and multiple randomly distributed thin-walled cysts with lower lobe predominance with patchy lobular air trapping consistent with lymphocytic interstitial pneumonia. Two-dimensional echocardiography revealed pulmonary artery systolic pressures of 35 mmHg. Spirometry showed restrictive pattern with forced vital capacity of 1.39 L (69%), forced expiratory volume in 1 s of 1.36 L (80%), and percentage of forced expiratory volume in 1 s out of forced vital capacity of 100%. Her 6 min walk distance was 370 m with significant postexercise desaturation. Fiber optic bronchoscopy along with transbronchial lung biopsy was done, and histopathological examination of biopsy samples revealed lymphocytic infiltrate confirming the diagnosis of lymphocytic interstitial pneumonia [Figure 3]. However, in view of the patient being relatively asymptomatic and the lung functions being well preserved, she was kept under observation with a regular 3-month follow-up.
|Figure 2: Computed tomography thorax showing multiple cysts and ground-glass opacities in bilateral lower lobe areas of lungs|
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|Figure 3: Microphotograph of transbronchial lung biopsy sample showing lymphocytic infiltration|
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| Discussion|| |
Interstitial lung disease is an assorted group of diseases of manifold etiologies sharing common clinical, radiological, and histological features. Recently, American Thoracic Society/European Respiratory Society have come up with a comprehensive revised classification of idiopathic interstitial pneumonias (IIPs) in 2013. As per this classification, IIPs are divided into three groups: (a) major IIP, (b) rare IIP and (c) unclassified IIP. Lymphocytic interstitial pneumonia is a benign lymphoproliferative disorder of lung characterized by exuberant and diffuse lymphocyte predominant infiltration of interstitium of the lung. LIP falls under the rare category as per the current classification. LIP was originally elucidated by Liebow and Carrington. It belongs to the broad spectrum of benign lymphoproliferative disorders: the other entities being intrapulmonary lymph nodes, foreign body, LIP, nodular lymphoid hyperplasia, and Castleman's disease. The entity of LIP may be seen in coexistence with a variety of conditions such as pediatric acquired immune deficiency syndrome, primary immunodeficiency, Sjögren's syndrome, myasthenia gravis, chronic liver disease, and dysproteinemic states. Majority of adults with LIP are women in the fourth to seventh decades of life  Idiopathic LIP is however immensely rare with only a handful of cases reported in literature till date. Diagnosis of LIP is made based on clinical and radiological findings and then confirmed through lung biopsy and subsequent histopathologic examination. The quotidian symptoms of lymphocytic interstitial pneumonitis include nonproductive cough and progressive exertional dyspnea of several weeks' duration. Some patients with LIP may have minimal symptoms or may be asymptomatic, and the diagnosis in such patients is only suggested by the finding of an abnormal CXR. Our patient also presented with only minimal respiratory symptoms, and an abnormal CXR instigated the further workup. Chest examination reveals bi-basal end-inspiratory crackles on auscultation. The CXRs of LIP patients vary and most often reveal fine (<3 mm) reticulonodular opacities, coarse reticulonodular opacities (3–5 mm), bibasilar diffuse infiltrates or consolidation. Our patient had fine reticulonodular opacities on CXR. HRCT findings are highly variable. Characteristic findings are centrilobular and subpleural nodules, thickened bronchovascular bundles, ground-glass opacities, cystic structures, interstitial septal thickening, and mediastinal lymphadenopathy. Our patient however showed the characteristic findings of cystic changes and ground-glass opacities on HRCT. Spirometry studies of LIP has revealed both a restrictive defect and a low diffusing capacity of the lung for carbon monoxide although obstructive pattern or normal spirometries have also been very rarely reported.
A definite diagnosis of LIP is based on lung biopsy findings, especially in the early stage. Microscopically, the LIP lung shows a predominantly interstitial cellular infiltrate that diffusely involves the distal parenchyma. The infiltrate is composed of a mixture of mature small lymphocytes, plasma cells, and histiocytes that widen the alveolar septa and surround small airways and vessels. Our patient was also diagnosed by a multimodality approach with radiological picture leading to a suspicion and the classical histological picture of interstitial lymphocytic infiltrate clinching the diagnosis. Hypersensitivity pneumonitis, nonspecific interstitial pneumonia, usual interstitial pneumonia, lymphoma of MALT, Pneumocystis jirovecii, fungal and mycobacterial infections, and number of lymphoproliferative disorders enter the differential diagnoses of LIP. Corticosteroids remain the mainstay of treatment with only limited randomized trials done in the territory. Other immunosuppressive agents such as cyclophosphamide, azathioprine, and colchicine are used only anecdotally with lack of clarity regarding their utility. Response to therapy is however quite variable. Our patient was kept under observation owing to her minimal symptoms and preserved lung functions. She thus was a noteworthy case of a rare disease with clement clinical presentation diagnosed only on the basis of her radiological and pathological findings.
| Conclusion|| |
Idiopathic LIP is rare as compared to LIP associated with conditions such as HIV or Sjogren's syndrome. Hence, its diagnosis warrants a high index of suspicion in light of the absence of such conditions. Opportune diagnosis can avoid irrevocable sequelae and save precious lives.
We would like to thank SRL laboratories.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]