desquamative interstitial pneumonia

Desquamative interstitial pneumonia is a chronic disorder that often has an insidious onset.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=591}} The most common symptoms of DIP are shortness of breath, especially during exercise, and coughing. Non-specific symptoms such as fever, weakness, weight loss, and fatigue are common. Other symptoms include respiratory failure, chest pain, digital clubbing, cyanosis, and rarely hemoptysis.{{sfn | American Journal of Respiratory and Critical Care Medicine | 2002 | p=296}} Some people with DIP may be asymptomatic, although this is rare.{{sfn | Godbert | Wissler | Vignaud | 2013 | p=117}}{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | pp=592-593}}

90% of desquamative interstitial pneumonia cases are linked to cigarette smoking. Other suggested causes of DIP include environmental or occupational exposure, systemic disorders and infections.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=588}} In many cases, no specific cause can be identified, and these are classified as idiopathic.{{sfn | Hellemons | Moor | von der Thüsen | Rossius | 2020 | p=8}}

Active or passive exposure to cigarette smoke is the most well-established risk factor for desquamative interstitial pneumonia. DIP has also been reported in those who do not smoke which indicates that there are other risk factors for DIP.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=588}}

Occupational exposure to diesel or fire smoke, asbestos, solder smoke, silica, beryllium, nylon filaments, wood dust, graphite, talc, copper dust, aluminum, and tungsten cobalt have been associated with DIP.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=588}}{{sfn | Godbert | Wissler | Vignaud | 2013 | p=120}}

DIP has also been associated with certain drugs such as marijuana, sirolimus, macrolides, nitrofurantoin, tocainide, and sulfasalazine.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=588}}

Several other disorders have been linked to DIP. Infections such as hepatitis C{{sfn|Iskandar|McKinney|Shah|Roy|2004|p=890}} and cytomegalovirus{{sfn|Sung|Ko|Kim|Kim|2005|p=637}} have been associated with DIP. Many systematic disorders such as systemic lupus erythematosus,{{sfn|Tubbs|Benjamin|Reich|McCormack|1977|p=163}}{{sfn|Kawabata|Takemura|Hebisawa|Ogura|2008|p=196}} connective tissue disease,{{sfn|Tubbs|Benjamin|Reich|McCormack|1977|p=163}}{{sfn|Lamblin|Bergoin|Saelens|Wallaert|2001|p=72s}} and rheumatoid arthritis{{sfn|Ishii|Iwata|Sakamoto|Mizunoe|2009|p=829}} have been connected to DIP.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | pp=588-589}}{{sfn | Godbert | Wissler | Vignaud | 2013 | p=121}}

Since laboratory testing, imaging, and bronchoalveolar lavage results are often non-specific, guidelines recommend surgical biopsy to diagnose desquamative interstitial pneumonia if high-resolution computed tomography does not reveal classic signs of interstitial pneumonia.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=590}} A definitive diagnosis of DIP relies on a lung biopsy.{{sfn | Godbert | Wissler | Vignaud | 2013 | p=119}}

File:Desquamative interstitial pneumonia (DIP) 2.jpg, many containing golden brown tobacco pigment.]]

While some laboratory abnormalities have been reported in cases of DIP, biological analysis does not usually point toward any diagnosis. Chest X-rays often show non-specific findings or come back normal.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=589}}{{sfn | Godbert | Wissler | Vignaud | 2013 | p=118}} Pulmonary function tests usually reveal a decrease in diffusion capacity and a restrictive pattern.{{sfn | Hellemons | Moor | von der Thüsen | Rossius | 2020 | p=8}}

Thoracic high-resolution computed tomography (HRCT) often shows signs of DIP,{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=590}} however, HRCT has only been reported on in one study. HRCT shows a ground-glass appearance.{{sfn | Godbert | Wissler | Vignaud | 2013 | p=118}}

The major hallmark of DIP is the presence of a large number of macrophages within the alveoli that are distributed throughout the pulmonary acini. These macrophages are rich in eosinophilic cytoplasm and frequently include a coarsely granular light-brown pigment. There are usually a few multinucleated large cells. The alveolar architecture is typically intact, however there is a modest chronic inflammatory infiltration inside the interstitium. A moderate quantity of eosinophils might also be present. Lymphoid aggregates can be present.{{sfn | Godbert | Wissler | Vignaud | 2013 | p=119}}

The differential diagnosis for DIP includes non-specific interstitial pneumonia, pulmonary Langerhans cell histiocytosis, respiratory bronchiolitis-associated interstitial lung disease, and hypersensitivity pneumonia.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | pp=589-590}}

The main treatment for DIP is quitting smoking. Sometimes smoking cessation is successful in managing DIP however in some cases this is not enough. Avoidance of other potential environmental factors is also advised. In those who are moderately to severely symptomatic and who have not responded to quitting smoking, corticosteroids are used. Cytotoxic and immunosuppressive drugs have been used for the treatment of DIP however there is not enough evidence on their usage. In cases of severe DIP lung transplants are an option however recurrence is still possible.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=593}}{{sfn | Godbert | Wissler | Vignaud | 2013 | p=121}}

Desquamative interstitial pneumonia has a favourable prognosis and most patients improve with proper treatment. The mortality rate of DIP is between 6 and 28%.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=593}} The survival rate of DIP is estimated to be between 68% and 94%. Without treatment around 60% of patients get worse. Spontaneous recovery had also been reported.{{sfn | Godbert | Wissler | Vignaud | 2013 | p=121}}

The prevalence of desquamative interstitial pneumonia is unknown,{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=588}} but it most commonly affects people aged 40 to 60,{{sfn | Hellemons | Moor | von der Thüsen | Rossius | 2020 | p=8}} with males twice as likely to have it as females.{{sfn | Godbert | Wissler | Vignaud | 2013 | p=117}} While DIP can sometimes progress rapidly, severe fibrosis is rare.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=588}}

Desquamative interstitial pneumonia was first defined by Averill Liebow et al. In 1965 Liebow described 18 patients with pulmonary lesions with large alveolar cell proliferation and desquamation. Liebow also noted that the walls of the patient's distal airways were thickened.{{sfn | Liebow | Steer | Billingsley | 1965 | pp=369, 371}} The name "desquamative interstitial pneumonia" originated from the assumption that the disease was caused by epithelial cell desquamation.{{sfn | Diken | Şengül | Coşkun Beyan | Ayten | 2018 | p=587}}{{sfn | Godbert | Wissler | Vignaud | 2013 | p=117}}

• Idiopathic interstitial pneumonia
• Respiratory bronchiolitis

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{{Medical resources

| ICD11 = {{ICD11|CB03.3}}

| ICD10 = {{ICD10|J84.1}}

| ICD10CM = {{ICD10CM|J84.117}}

| ICD9 = {{ICD9|516.37}}

| ICDO =

| OMIM = 263000

| MeshID = C562470

| DiseasesDB = 33796

| SNOMED CT = 8549006

| Curlie =

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| eMedicineSubj =

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| PatientUK =

| NCI =

| GeneReviewsNBK =

| GeneReviewsName =

| NORD =

| GARDNum =

| GARDName =

| RP = 6537

| AO =

| WO =

| OrthoInfo =

| Orphanet = 98852

| Scholia = Q5265029

| OB =

}}

{{Respiratory pathology}}

Category:Histopathology

Category:Pneumonia