Ein case report: Positive klinische Entwicklung einer seltenen Erkrankung (GPCR-Autoantikörper (AABs), vermittelte Mikroangiopathie mit idiopathischer Hypoxämie) durch Immunadsorption (IA)
About 3 years ago, the 67-year old male patient complained of CHD typical symptoms, but more likely at the beginning or after exercise. Cardiological and pulmonological examinations showed no pathological features.
In the meantime, clinical signs of hypoxemia (lip cyanosis, acrocyanosis) and evidence of strongly fluctuating decreased oxygen saturation up to 90% were observed.
In addition, there were also new symptoms of ME/CFS and GPCR-AABs were detected in Bioassay and Elisa technologies.
After screening for eligibility the patient received 5 IA-treatments on 5 consecutive days (primary device: Spectra Optia, Terumo BCT; secondary device: ADAsorb medicap clinic with TheraSorb Ig omni 5 Adsorber, Miltenyi Biotec B.V.&Co. KG). The primary endpoint was the removal of AABs. The treatment resulted in lowering of plasma IgG to levels below 2 g/l with focus on the elimination of AABs.
The removal of IgG was successfully high (IgG lowering about 90%). The control immediately after the IA showed no more AABs detectable.
The first therapy with 5 sessions in January 2023 resulted without improvement of symptoms. After the 2nd series of IA in May 2023 (5 treatments on 5 consecutive days) the patient recognized a slow improvement and no more AABs were detected in Bioassay.
The general condition has improved quite significantly: still dependent on oxygen, but with oxygen administration hardly any pain, no more continuous bedriddenness quite acceptable at rest, but almost no resilience and significantly less stenocardia and less dyspnea.
Based on the highly probable classic diagnosis of idiopathic hypoxemia, there is a suspicion that the condition is not idiopathic, but an AAB-mediated microangiopathy. The AABs would then also be responsible for the other symptoms described at the beginning.
IA resulted in a significant reduction of symptoms of our patient and none worsened.
In conclusion this clinical picture is unknown, because idiopathic hypoxemia itself is extremely rare, and AABs have never been discussed in this context.
None.