Ceelen-Gellerstedt syndrome
| Classification according to ICD-10 | |
|---|---|
| E83.1 | Iron metabolism disorders |
| ICD-10 online (WHO version 2019) | |
The Ceelen-Gellerstedt syndrome is a rare lung disease. Synonym: Idiopathic primary hemosiderosis of the lungs.
Cause and epidemiology
The origin of this rare disease is unclear. An autoimmune process is assumed, i.e. an overreaction of the body's own immune system . An increased simultaneous occurrence of cow's milk allergy ( lactose intolerance , Heiner syndrome ) and gluten intolerance ( celiac disease , Lane-Hamilton syndrome ) was described. One can differentiate between a chronic form with onset in childhood and familial accumulation and a rare acute form in adulthood. It mainly affects men in a ratio of 2: 1.
pathology
The disease leads to damage to the smallest blood vessels in the lungs. This leads to recurring small but also larger bleeding into the lung tissue. These repeated damage to the lung tissue, v. a. Due to the deposits of the blood breakdown product hemosiderin , gradually lead to an increased production of connective tissue and thus to pulmonary fibrosis .
Symptoms
The symptoms depend on the activity of the disease, i. H. the number of episodes of bleeding and the extent of the bleeding and, in the later stages, the extent of the fibrosis. In general, the bleeding leads to hemoptysis ; H. for coughing up blood, and for anemia ( anemia ) with all its symptoms such as dyspnea (shortness of breath), tachycardia (rapid heartbeat), weakness and tiredness. If the coughed up blood is swallowed, it can also lead to vomiting of blood ( hematemesis ). With the increasing development of pulmonary fibrosis , shortness of breath comes more and more to the fore.
diagnosis
The suspected diagnosis is made based on the symptoms. A further clarification is carried out by means of imaging procedures ( X-ray and computer tomography ). If there is a specific suspicion, the diagnosis is confirmed using tissue samples from the lungs by means of fine-tissue examinations. Alternative diagnoses that must be taken into account with the symptoms described are vasculitides (inflammation of the vessels) and secondary hemosiderosis of the lungs in the case of heart valve defects ( mitral vitia ).
Therapy and prognosis
An effective causal therapy does not yet exist. The prognosis for the disease is poor. Up to 10% of sick children die within the first year of diagnosis. The 12-year survival rate is approximately 40%.
swell
- A. Fendel, J. Tremper, M. Horger: Idiopathic hemosiderosis of the lung, synonym is Ceelen-Gellerstedt syndrome. In: Rofo. 2002 Jun; 174 (6), pp. 653-635.
