General measures In respiratory insufficiency (disruption of pulmonary gas exchange), long-term oxygen therapy is given (see below). If necessary, intensive medical measures in case of increasing respiratory distress. Review of permanent medication due topossible effect on the existing disease. Travel recommendations: Participation in a travel medical consultation required! Air travel only with an additional oxygen … Pulmonary Fibrosis: Therapy
The following are the most important diseases or complications that may be contributed to by pulmonary fibrosis: Respiratory system (J00-J99) Respiratory insufficiency (limitation of breathing)-increased pressure in the pulmonary arterial system with any of the following: Arterial partial pressure of oxygen <70 mmHg during spontaneous breathing. Horrowitz index < 175 mmHg (oxygenation index; paO2/FiO2). Hyperventilation … Pulmonary Fibrosis: Complications
For a diagnosis of idiopathic pulmonary pulmonary fibrosis (IPF), either criteria 1 and 2 or 1 and 3 must be met: Interstitial lung disease (ILD) or diffuse parenchymal lung disease (DPLD) of known cause (e.g., noxious exposure, collagenoses, other systemic diseases, drug-induced ILD, etc.) must be excluded. In high-resolution computed tomography (HRCT), a UIP pattern … Pulmonary Fibrosis: Classification
A comprehensive clinical examination is the basis for selecting further diagnostic steps: General physical examination – including blood pressure, pulse, body weight, height; further: Inspection (viewing). Skin, mucous membranes, and sclerae (white part of eye) [central cyanosis (bluish discoloration of skin and central mucous membranes, e.g., tongue), drumstick fingers, watch glass nails] Auscultation (listening) of … Pulmonary Fibrosis: Examination
2nd-order laboratory parameters-depending on the results of the history, physical examination, and obligatory laboratory parameters-for differential diagnostic workup Small blood count Differential diagnosis Inflammatory parameter – CRP (C-reactive protein) Rheumatoid factor Cyclic citrulline peptide antibodies (CCP-AK) Antinuclear antibodies (ANA) Blood gas analysis (BGA) Histology (fine tissue examination), cytology (cell examination) of biopsies (tissue sampling) taken … Pulmonary Fibrosis: Test and Diagnosis
Therapeutic target Halting progression (progression of the disease). Therapy recommendations Therapy depends on the underlying disease. Therapy of idiopathic pulmonary fibrosis (idiopathic pulmonary fibrosis, idiopathic pulmonary fibrosis (IPF)/idiopathic pulmonary fibrosis) is usually with prednisiolone (glucocorticoids); furthermore, immunosuppressants (e.g., azathioprine) are used. IPF guideline 2015: prednisone + azathioprine + N-acetylcysteine; strong recommendation against (PANTHER; IPF guideline … Pulmonary Fibrosis: Drug Therapy
Obligatory medical device diagnostics. Pulmonary function diagnostics [objectification of restrictive ventilation disorder and gas exchange disorder]. Spirometry Whole-body plethysmography Diffusion measurement X-ray of the thorax (X-ray thorax/chest), in two planes. Computed tomography of the thorax/chest (thoracic CT); preferred here: high-resolution computed tomography (HRCT; slice thickness ≤ 2 mm); without contrast medium administration [idiopathic pulmonary fibrosis … Pulmonary Fibrosis: Diagnostic Tests
To prevent pulmonary fibrosis, attention must be paid to reducing individual risk factors. Behavioral risk factors Drug use Cocaine Inhalation of noxious agents (tobacco smoke + other noxious agents: see below “Environmental pollution – Intoxications”); but does not occur primarily in smokers; however, former or active smokers have an overall 1.6-fold higher risk Drugs (including … Pulmonary Fibrosis: Prevention
The following symptoms and complaints may indicate pulmonary fibrosis: Clinical symptoms begin insidiously and consist of: Increasing exertional dyspnea (shortness of breath on exertion). Tachypnea – excessive respiratory rate. Dry cough / irritable cough (= irritable cough without sputum). In the further course Resting dyspnea (shortness of breath at rest). Drumstick fingers – thickening of … Pulmonary Fibrosis: Symptoms, Complaints, Signs
Pathogenesis (disease development) Pulmonary fibrosis is a group of chronic diseases in which there is remodeling of the lung skeleton (increase in connective tissue). In idiopathic pulmonary fibrosis (idiopathic pulmonary fibrosis, idiopathic pulmonary fibrosis IPF), apoptotic alveolar epithelia likely results in the following processes: Disruption of regeneration Activation of fibroblasts (main component of connective tissue). … Pulmonary Fibrosis: Causes
Medical history (history of illness) represents an important component in the diagnosis of pulmonary fibrosis. Family history What is the general health of your family members? Are there any diseases in your family that are common? Social history What is your profession? Are you exposed to harmful working substances in your profession? Current medical history/systemic … Pulmonary Fibrosis: Medical History
Congenital malformations, deformities, and chromosomal abnormalities (Q00-Q99). Hermansky-Pudlak syndrome – genetic disorder with autosomal recessive inheritance associated with albinism, photophobia, and increased bleeding tendency; usually also pulmonary fibrosis and increased bleeding tendency. Neurofibromatosis – genetic disease with autosomal dominant inheritance; belongs to the phakomatoses (diseases of the skin and nervous system); three genetically distinct forms … Pulmonary Fibrosis: Or something else? Differential Diagnosis