Sarkoidose

Wessendorf, Thomas; Bonella, Francesco; Costabel, Ulrich

doi:10.1007/s10405-012-0665-0

Cited by 3 publications

(2 citation statements)

References 25 publications

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“…Verkalkungen der hilären und medistinalen Lymphknoten kommen vor. Sonderformen der Lungensarkoidose sind noduläre Formen sowie kavitäre Formen, vor allem fibrös-zystisch in den Oberlappen lokalisiert, mit der gefürchteten Komplikation einer Besiedelung mit Aspergillomen [14].…”

Section: Pulmonale Manifestationunclassified

“…Eine bronchiale Überempfindlichkeit lässt sich bei bis zu 30 % der Patienten mit Lungensarkoidose nachweisen, nicht selten mit hartnäckigem trockenem Husten verbunden. Bei Belastungsdyspnoe ohne plausible Klärung durch entsprechende schwere Funktionsstörungen sollte auch immer an eine pulmonale Hypertonie gedacht werden [14,18]. Nach der Dana-Point-Klassifikation wird sie der Gruppe V (unklarer oder multifaktorieller Mechanismus im Rahmen einer systemischen Erkrankung) zugeordnet, sofern nicht eine schwere restriktive Ventilationsstörung vorliegt (dann Gruppe III).…”

Section: Pulmonale Manifestationunclassified

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Epidemiologie und klinisches Erscheinungsbild der Sarkoidose

Costabel¹,

Wessendorf²,

Bonella³

2016

Klin Monatsbl Augenheilkd

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Sarcoidosis is a systemic disease of unknown aetiology. Typical histology shows epithelioid cell granulomas, and typical immunopathology enhanced Th1 type immune responses in the involved organs. The disease occurs worldwide, but more frequently in northern countries than in the south. In Germany, the incidence is estimated to be 10 per 100,000, and the prevalence 44-48 per 100,000. Sarcoidosis usually affects adults under 50 years of age, but can also be seen in children, adolescents and in the elderly. Women are more frequently affected than men. Familial clusters can occur. The clinical presentation of sarcoidosis varies widely and depends on the manifestations in the individual organ. Systemic symptoms include fatigue, night sweats, weight loss, fever, arthralgia and myalgia. Organ-specific symptoms include cough and dyspnoea, with pulmonary involvement, headache and palsy in neurosarcoidosis, arrhythmias and heart failure in cardiac sarcoidosis, and manifold skin lesions with skin involvement. Relapses are rarely seen in acute sarcoidosis, whereas the chronic form tends to relapse more frequently. Löfgren's syndrome, a specific phenotype of acute sarcoidosis, is characterised by bihilar lymphadenopathy, ankle arthritis and erythema nodosum. Chronic sarcoidosis can be asymptomatic, despite radiological changes, which may be extensive. By definition, sarcoidosis has become chronic after 2 years of disease with ongoing signs of activity. The long-term prognosis is generally good, but depends on the different organ manifestations and complications.

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Section: Pulmonale Manifestationunclassified

Epidemiologie und klinisches Erscheinungsbild der Sarkoidose

Costabel¹,

Wessendorf²,

Bonella³

2016

Klin Monatsbl Augenheilkd

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Therapeutische Relevanz des HRCT-Befundes aus pneumologischer Sicht

Suchy

Pfeifer

2014

Radiologe

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The high-resolution computed tomography (HRCT) technique is an essential component in diagnosing interstitial lung disease (ILD) as it provides important and specialized information and a much greater accuracy than chest X-rays. It contributes to a narrowing down of the differential diagnoses and is also important for planning further invasive investigations, e.g. bronchoscopy, bronchoalveolar lavage, transbronchial lung biopsy and surgical lung biopsy, if needed. An accurate diagnosis of ILD is based on a multidisciplinary discussion involving pulmonologists, radiologists and pathologists experienced in the diagnosis of ILD.The therapy approaches of five different entities of ILD are shown as examples. In hypersensitivity pneumonitis the mainstay of treatment is complete avoidance of exposure to the provoking antigen. In cryptogenic organizing pneumonia most patients recover with corticosteroid therapy with prednisolone over a period of 6 months to 1 year. In cases of sarcoidosis therapy depends on organ involvement and functional impairment but there is no durable benefit to routine treatment of patients with acute pulmonary sarcoidosis, even among those with stage II or III chest radiographs. In general, patients with severe or progressive disease will require treatment.In idiopathic pulmonary fibrosis (IPF) a confident radiological diagnosis of definitive usual interstitial pneumonia (UIP) obviates the need for surgical lung biopsy. Other etiologies of a HRCT pattern of UIP, such as domestic and occupational environmental exposure, connective tissue disease and drug toxicity must be ruled out. In IPF antifibrotic therapy with pirfenidone (approval since 2011) or the triple tyrosine kinase inhibitor nintendanib (pending approval in 2015) can reduce disease progression but therapy with acetylcysteine alone or in combination with prednisolone and azathioprine failed to meet efficacy endpoints. In the management of scleroderma associated ILD rapid semiquantitative evaluation of the extent of the disease on HRCT > 20% is useful for identifying patients who require treatment.

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Interstitial pulmonary diseases

Costabel

2013

Pneumologe

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Sarkoidose

Cited by 3 publications

References 25 publications

Epidemiologie und klinisches Erscheinungsbild der Sarkoidose

Epidemiologie und klinisches Erscheinungsbild der Sarkoidose

Therapeutische Relevanz des HRCT-Befundes aus pneumologischer Sicht

Interstitial pulmonary diseases

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