Brief Answer:
CVID leads to recurrent infections.
Detailed Answer:
Hi,
Thanks for posting the query, on XXXXXXX Since the query has been directed to me, I would like to give my opinion on it from Pulmonologist point of view. After going through your query, I would like to comment the following:
1. You have been worked up in detail and have been diagnosed with a lot of comorbidities and your symptom complex is a result of interaction of different disease processes in your body.
2. To summarise your diagnosis you seem to be suffering from CVID (Common Variable Immuno Deficiency Syndrome) with Psoriasis with gout with Arthritis (Psoriatic vs gouty) with Obesity with
Obstructive sleep Apnea.
3.Most of your co morbidities are interlinked. Obesity and Psoriasis are the two risk factors that may cause
Increased uric acid in your body (Gout). Gout and Psoriasis are two risk factors of arthritis in your body. Obesity is the single most risk factor of Obstructive sleep apnea (OSA) especially fat deposition rounf the neck in view of your history of neck swelling. There could be an additional component of
obesity hypoventilation syndrome (ABG analysis for PaCO2 will help here). Memory issues can again be linked to OSA. Autoimmune phenomenon is known in persons with known allergies like you eg.
penicillin allergy.
4. Coming to your respiratory system. You have been diagnosed with CVID. CVID is very well known to cuase recurrent respiratory tract infections which have led to your repeated episodes of coughing up blood. The recurrent infections associated with with CVID can be in a structurally normal lungs and hence the XXXXXXX ct scans. However the bronchoscopy showed inflammation and that signifies that there is inflammation of your respiratory lining epithelium which is very much dependant on immunoglobulins. There may be some ciliary motility issues associated with the inflammation of bronchi which leads to pent up secretions in the bronchi during early stages of infection. The point is to prevent these infective episodes since repeated infections will ultimately lead to development of bronchiectasis.Sterois and IV
immunoglobulin therapy may be of help in reducing the inflammation. Prophylactic azithromycin will help serve the aim of preventing respiratory infection. The efficacy of pneumococcal vaccinations is doubtful in CVID hence vaccination will not be an effective option to be relied upon in this condition due to the deficient anti body production.
5. Since you have an enlarged liver with early hypoxemia,
hepatopulmonary syndrome should be ruled out by seeing any postural fall of oxygen saturation.
6. For your obstructive sleep apnea , are you on CPAP? By ruling out asthma, I suppose your spirometry was normal? What are your serum IgE levels?
7. I suppose Extranuclear antigen profile for collagen vascular diseases has been done and is negative. Sarcoidosis and chronic hypersensitive pneumonitis must have been ruled out. ECG, 2D ECHO and stress test should have ruled out any cardiac involvement.
8. Possible causes for postural dizziness are local (ENT) eg meniere's disease, Cardiac or postural hypoxemia.
9. Associated asbestos exposure is a risk factor for such airway inflammation. Are you on any inhalers? Have you ever smoked.
10. Coming to your
exertional dyspnea relieved on exercise, I would like you to undergo a full cardipulmonary exercise testing /if not possible, then a simple six minute walk test. Causes of exertional dyspnea relieved on exercise are commonly GERD (acidity), anxiety or panic.
11. Coming to your shunt test. Normal shunt fraction is less than 5 percent. The shunt tests are normally designes to quantify the shunt rather than localise it. The shunt fraction helps us know the fraction of blood escaping oxygenation.Common cause is consolidation,rarely, hepatopulmonary syndrome and AV malformation.
12. It most likely seems that on exercising, the warm air helps relieve the inflammed airways and hence helps you feel better. You may try home remedy with warm moist air inhalation from hot water to see if it helps. Such a phenomenon is sometimes seen in asthmatics.
I hope I have answered your query. I will be glad to answer follow up queries if any.
Please accept my answer if you have no follow up queries.
Regards
Dr. Gyanshankar Mishra
MBBS MD DNB
Consultant Pulmonologist