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Acute Interstitial Pneumonitis

Hamman-Rich syndrome


Presentation

  • A 15-year-previously-healthy male boy presented to the Hospital with a 6-day history of malaise, fever and cough.[ncbi.nlm.nih.gov]
  • We present a case of acute interstitial pneumonitis in a patient who was treated with hydroxyurea for essential thrombocythemia. The clinical course suggests that the interstitial pneumonitis was induced by hydroxyurea.[ncbi.nlm.nih.gov]
  • A 14-year-old girl with skin rash and acute interstitial pneumonitis is presented. She had been taking carbamazepine for two months.[ncbi.nlm.nih.gov]
  • We report a case of anti-synthetase syndrome presenting as acute interstitial pneumonia (AIP). CASE PRESENTATION We present the case of a 42-year-old school teacher of Malaysian origin. She was an ex-smoker.[academic.oup.com]
  • A review of previous and present studies of azacitidine-associated AIP suggests that azacitidine may cause life-threatening AIP during or after the first seven-day course of treatment, with pyrexia commonly preceding AIP.[ncbi.nlm.nih.gov]
Fatigue
  • A person may have these symptoms: A dry cough Shortness of breath, which may occur after effort or become worse over time Difficult or labored breathing Fatigue Clubbing, or enlargement of the fingertips at the base of the nails.[my.clevelandclinic.org]
  • Oxygen therapy Because many people with ILD have low levels of oxygen in their blood, oxygen therapy improves blood oxygen levels while reducing strain on the heart and lungs and improving breathlessness and fatigue. [5] Pulmonary rehabilitation A formal[inogen.com]
  • Patients may also experience fatigue and myalgia. Acute respiratory failure develops in previously healthy individuals without pre-existing lung disease. The signs and symptoms of AIP include tachypnoea, dyspnoea, cyanosis, crackles and wheezes [2].[ncbi.nlm.nih.gov]
  • Symptoms which chronic pneumonitis may lead to: fatigue, weight loss, exercise intolerance, cyanosis and finger clubbing.[patient.info]
Chronic Dermatitis
  • Skin biopsy confirmed psoriasiform erythroderma with chronic dermatitis. HRCT done 4 weeks later showed complete resolution of the previous findings.[jfmpc.com]
Hyperkeratosis
  • Our patient had an atypical presentation of anti-synthetase syndrome; she lacked evidence of Raynaud phenomenon, hyperkeratosis and arthritis. She also developed the less commonly associated ILD pattern, AIP.[academic.oup.com]
Difficulty Concentrating
  • concentrating, forgetfulness) because of long-term reduced oxygen supply Sudden onset of high fever Persistent cough that won’t go away Acute Interstitial Pneumonia (AIP) Overview: TREATMENT Treatment is supportive and usually requires mechanical ventilation[healtheo360.com]
  • concentrating, forgetfulness) because of long-term reduced oxygen supply Diagnosis and Treatment of Acute Interstitial Pneumonia We value and respect our HERWriters' experiences, but everyone is different.[empowher.com]

Workup

  • Initial workup revealed ground-glass opacities on computed tomography (CT) scan of the chest. Cardiac workup was unrevealing with a normal myocardial perfusion stress test.[ncbi.nlm.nih.gov]
  • […] can also be found in other diseases such as CTDs, which is another ILD classification, but is idiopathic when the cause is unknown despite a comprehensive workup.[consultant360.com]
  • […] often needed to make a diagnosis, with pertinent information and expertise contributed by the pulmonologist, radiologist and pathologist. 4 High-resolution CT should be obtained in virtually all patients with a suspected ILD. 4 Additional diagnostic workup[appliedradiology.com]
  • BAL cell count and differential may be useful in the diagnostic workup of the hospitalized patient with ILD.[bmcmedicine.biomedcentral.com]
Alveolar Consolidation
  • HRCT 20, 21 proved to be nonspecific, by showing bilateral areas of ground glass attenuation and/or diffuse alveolar consolidation.[erj.ersjournals.com]
  • Chest radiograph [Figure 3] and High-resolution computerized tomography (HRCT) of the thorax showed extensive multilobar alveolar consolidation. His antibiotics and antifungals were stopped after the blood and sputum cultures remained sterile.[jfmpc.com]
  • HRCT scans show bilateral and symmetric, diffuse ground glass and alveolar consolidation opacities. Traction bronchiectasis and bronchiolectasis appear as the disease progresses. Honeycomb fibrosis may be seen in the later phase of the disease.[emedicine.medscape.com]
Air Bronchogram
  • Air bronchograms were present.[ncbi.nlm.nih.gov]
  • Laboratory Hypoxia Increased serum ferritin, D dimer and C reactive protein KL-6 may increase slightly Radiology description Heterogeneous bilateral ground glass opacity due to pulmonary edema Chest radiograph Ground glass opacity Consolidation with air[pathologyoutlines.com]
Left Pleural Effusion
  • There is a small left pleural effusion. CXR Suboptimal aspiration with small volume lungs. There is consolidation in the right mid zone and left lower lobe. Small bilateral pleural effusions are present.[ncbi.nlm.nih.gov]

Treatment

  • This report describes a case of MDS in a patient who developed fatal acute interstitial pneumonitis (AIP) after the first seven-day course of intravenous azacitidine (75 mg/m(2)/day) treatment.[ncbi.nlm.nih.gov]
  • The treatment of EGFR-TKI agents induced acute hepatitis remains uncertain and cessation medication is current policy.[ncbi.nlm.nih.gov]
  • The only treatment that has met with success to date is a lung transplant. Acute interstitial pneumonitis occurs most frequently among people older than forty years old. It affects men and women equally.[en.wikipedia.org]
  • Because it is typically diagnosed at an advanced stage, chemotherapy (CT) remains the cornerstone of treatment, however, conventional treatment of NSCLC has apparently reached a plateau of effectiveness in improving survival of patients.[jtd.amegroups.com]
  • A literature review found four similar cases of infliximab-induced interstitial lung disease in the setting of treating patients with UC using standard infliximab regimens had successful treatment of the subjects post infliximab discontinuation.[ncbi.nlm.nih.gov]

Prognosis

  • Unfortunately, patients with polymyositis/dermatomyositis associated with pulmonary complications have a worse prognosis than patients with isolated forms.[ncbi.nlm.nih.gov]
  • It has a grave prognosis with 70% mortality in 3 months, despite mechanical ventilation. This review aims to clarify the relative clinical and pathological issues and terminology.[ncbi.nlm.nih.gov]
  • Prognosis depends on the histopathology findings and the specific form of interstitial lung disease and its response to therapy, which consists of high-dose steroids and immunomodulating agents.[rc.rcjournal.com]
  • Even despite mechanical ventilation, it often carries a grave prognosis with 70% mortality at 3 months 1.[radiopaedia.org]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • […] specific etiologic agent or inciting event.[emedicine.medscape.com]
  • Katzenstein reserves the term AIP for those cases where there is no identifiable underlying etiology (such as sepsis).[histopathology-india.net]
  • This condition, now popularly known as the Hamman-Rich syndrome, is generally considered to be of unknown etiology, although various theories have been propounded.[nejm.org]
  • Hamman-Rich syndrome became synonymous with idiopathic pulmonary fibrosis despite clear differences in clinical presentation, radiography, pathology, and survival.[ 3 ] In 1986, Katzenstein described eight patients with acute respiratory failure of unknown etiology[thieme-connect.com]

Epidemiology

  • Many questions still linger regarding the epidemiology, pathogenesis, and outcome.[thieme-connect.com]
  • Extremely rare (no conclusive epidemiological data available) Mean age 50 years but can occur at any age (7 - 83 years) ( Eur Respir J 2000;15:412 ) No sex predilection Sites Bilateral lung, usually in all five lobes of the lung Pathophysiology Both[pathologyoutlines.com]
  • […] acute respiratory distress syndrome (ARDS) attributable to an identifiable cause, as well as patients with underlying fibrotic lung disease or systemic disorders known to be associated with lung involvement such as connective tissue disease. [1, 2] Epidemiology[emedicine.medscape.com]
  • “Invasive aspergillosis: Epidemiology, diagnosis and management in immunocompromised patients”. Drugs. vol. 67. 2007. pp. 1567-601.[infectiousdiseaseadvisor.com]
  • Epidemiology It is common, if all causes of pneumonitis are considered.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • We describe the case of a fulminant form of AIP and discuss the pathophysiological mechanisms of AIP with reference to the histological pattern.[ncbi.nlm.nih.gov]
  • […] damage Epidemiology Extremely rare (no conclusive epidemiological data available) Mean age 50 years but can occur at any age (7 - 83 years) ( Eur Respir J 2000;15:412 ) No sex predilection Sites Bilateral lung, usually in all five lobes of the lung Pathophysiology[pathologyoutlines.com]
  • Pathophysiology [ edit ] Macklin described the pathophysiology of Hamman's syndrome to be barotrauma, whereby rupture of alveolar membranes causes a positive pressure gradient of air from the lungs into the mediastinum (the Macklin effect). [3] Diagnosis[en.wikipedia.org]
  • Eosinophilic pneumonia Eosinophilic pneumonia is similar to organizing pneumonia in pathophysiology, but is mediated instead by an inappropriate increase in the number of eosinophils and macrophages in alveoli.[appliedradiology.com]
  • Pathophysiology NA Epidemiology The overall incidence of acute respiratory failure in patients with ILD has not been well established. Infection and acute exacerbations of underlying ILD appear to be the most common precipitating etiologies.[clinicaladvisor.com]

Prevention

  • Further study to identify causal factors is required in the hope of preventing morbidity and mortality related to this disease.[ncbi.nlm.nih.gov]
  • After a few days of adjuvant therapy with temozolomide and prophylaxis with trimetrophin-sulfamethoxazolo to prevent Pneumocystis Jiroveci, she had progressive and rapid worsening of symptoms with weakness, dyspnea and orthopnea.[currentdrugsafety.com]
  • To effectively follow up patients treated with gefitinib and to prevent deaths from gefitinib-induced lung injury, early recognition and intervention may prevent a fatal outcome, corticosteroids and non-invasive BIPAP-ventilation should be considered[jtd.amegroups.com]
  • This has helped prevent admission to ITU for intubation with a positive effect on acute hypoxemic respiratory failure and secretions management.[ncbi.nlm.nih.gov]
  • Although pathological manifestation may vary from one case of AIP to another, our finding partly indicated the possibility and importance of EMT in AIP and provided a potential therapeutic method of preventing EMT in AIP.[link.springer.com]

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