question-icon

What causes symptoms of Sicca syndrome along with fatigue and lymphadenopathy

default
Posted on Sat, 14 Mar 2015
Question: In 2004 I had trochenteritt and auto-antibodies for SSA and CENP-B was discovered. 2 years ago my mother was diagnosed with systemic vasculitis and temporal artritis. The years since then I have had big problems with huge fatigue, lunge infections, joint pain and brown pigment spots in my face. The last 6 months after an infection I felt like I was dying. At some days I could not even manage to take a shower because of extreme fatigue. I slept 12-15 hours each day. I went through an rheumatic report 2 weeks ago and was diagnostified with Sjøgrens Syndrome based upon Sicca-symptoms, swullen lymph glands, anti-SSA 240. Raynaud since childhood but it has been much worse last 2 years. I started with Plaquenil. My problem is that I also have a positive anticentromer (CENP-B 166). My rheumatologist says I dont have limited systemic sclerosis now but there is a chance I will develop that in the future. He says that if you have one connective tissue disease you are likely to have one more. CENP-B is not common in healthy people right? Can it be because of my Raynaud alone without getting more symptoms for sclerosis? I don't have lupus or autoimmun hepatitis. How big percentage chance is there I will develop limited systemic sclerosis? I am very conserned about this. Will this always start with swullen hands? i also wonder based upon my medical history if I might have had Sjøgrens since childhood but with sicca as a late debut (last years)? Please comment upon my history and be spesific for my questions. Thank you!
doctor
Answered by Dr. Naval Mendiratta (4 hours later)
Brief Answer:
? Is it A mixed Connective tissue disease

Detailed Answer:
Good evening

Thank you for writing on health care magic

I have gone through your history. Well the symptoms of sicca along with fatigue and lymphadenopathy along with SSA positive do fit into sjogrens syndrome. But yes, the diagnosis can only be confirmed by a positive salivary gland biopsy as most of these connective tissue diseases are clinically diagnosed.

Pertaining to your queries:

1. CENP B is quite a specific antibody for limited scleroderma. It co relates very well with your raynauds although raynauds itself can present along with Sjogrens syndrome. It is more like a symptom than a conclusion. But if CENP B is positive, it goes high in favor of limited systemic sclerosis. No, it is not common in healthy people

2. Yes, you can have raynauds for another few years before any symptoms of sclerosis develop Exact duration, one cant predict. But what you need to understand for the disease is that it is a very slow progressive. It can take up to 10-15 years before any further symptoms develop. Exact percentage one cant predict, but studies say 20-30 %.

3. As for CENP B positive, the most common problem is raynauds. Later patients develop skin tightening which is limited to fingers and feet. The next common thing you need to monitor is your Heart Pressures. Once a year 2d Echo to asses for pulmonary ARtery PRessure has to be done. Lungs and kidneys are less common of the involvement in CENP B positive patients

4. I doubt you would have sjogrens since childhood. Some event has happened in between which has triggered the antibody formation in you and caused these symptoms.

HOpe the information has been useful

DO let me know for more queries. Would be happy to help out

Regards
Dr Naval
Above answer was peer-reviewed by : Dr. Yogesh D
doctor
default
Follow up: Dr. Naval Mendiratta (7 hours later)
Thank you very much for your answer.

Why do you think this might be MCTD instead of Sjøgrens Syndrome (SSA) with a secondary CTD (CENP-B)?

Instead of a salivary gland biopsy they took a ultrasound of major salivary glands that showed atrophy. Schirmer test 0 mm!

So when I have a positive CENP-B along with Raynaud it will be 100% sure that I will develop limited systemic sclerosis?

Is it not common that limited systemic sclerosis affect the skin in the face and the lungs?

Why is my lymph glands so cronically swullen all the time?

What is the best medication for Sjøgren? Especially for the fatigue since this is my biggest problem. Will Plaquenil make the progression of Limited sclerosis even slower?

doctor
Answered by Dr. Naval Mendiratta (8 hours later)
Brief Answer:
Sjogrens Syndrome

Detailed Answer:
Good evening

Well there is no such terminology as a secondary CTD. We use it for only Sjogrens ( Primary vs Secondary). It can very well be an overlap between Sjogrens and systemic Sclerosis. ( Overlap Syndrome)

2. If the schirmmer is 0 as well along with salivary gland atrophy, It definitely confirms Sjogrens Syndrome.

3. Not necessarily that you will develop full blown limited systemic Sclerosis. As i mentioned earlier, just antibody positivity does not imply you are gonna have a full blown disease. It gives us an implication on what we need to monitor in due course of time. Limited Systemic Sclerosis does affect the lungs but in a very late stage. The skin involvement is usually limited to hands( upto elbows) and the feet with rare involvement of face. There is never a kidney involvement in it. But the disease progression is very slow and one is never sure when you will develop the symptoms.

4. Your swollen lymph glands are probably due to Sjogrens. Never the less, if they havent done a Biopsy for it yet, i would recommend you to get it done.Just to be sure it is a non specific enlargement due to sjogrens and not any other condition which we are missing.

5. As for the medication, Plaquenil is a very good drug but no it wont stop the progression of limited sclerosis.We never use it for Limited Sclerosis either. But if the lymph nodes are quite swollen, we tend to use a low dose of steroids. It will help your fatigue and the swollen glands as well.
Also, you should discuss with your rheumatologist about the B cell therapy( Rituximab). Considering Sjogrens and Systemic Sclerosis as both originator from the B Cells, Rituximab has recently been used in the early stages to halt the progression of the disease. It is still not FDA approved, but lot of research work is going on for it and has shown good results.

Hope the information was useful

Do let me know about more queries

Would be happy to help you out

Regards
Dr Naval
Above answer was peer-reviewed by : Dr. Vinay Bhardwaj
doctor
default
Follow up: Dr. Naval Mendiratta (29 hours later)
Thank you very much for your helpful answeres of good quality! I will discuss some of the things you mentioned with my rheumatologist.

One last question: I have brown pigment spots in my face (comes especially after sun exposure). Do you think it has something to do with Connective tissue disease?

Thanx a lot!!:))
doctor
Answered by Dr. Naval Mendiratta (4 hours later)
Brief Answer:
it can be a part of it

Detailed Answer:
Good evening

Well, it can be a part of it. A lot of pigmentation and photosenstivity which are unexplained can accompany the Connective tissue disease. Although such spots are more common with SLE, but can be seen in any connective tissue disease. Try using a good sunscreen lotion( with SPF 50). That should help control these photosenstive spots

Do let me know for more queries

Regards
Dr Naval
Above answer was peer-reviewed by : Dr. Shanthi.E
doctor
Answered by
Dr.
Dr. Naval Mendiratta

Rheumatologist

Practicing since :2007

Answered : 754 Questions

premium_optimized

The User accepted the expert's answer

Share on
What causes symptoms of Sicca syndrome along with fatigue and lymphadenopathy

Brief Answer: ? Is it A mixed Connective tissue disease Detailed Answer: Good evening Thank you for writing on health care magic I have gone through your history. Well the symptoms of sicca along with fatigue and lymphadenopathy along with SSA positive do fit into sjogrens syndrome. But yes, the diagnosis can only be confirmed by a positive salivary gland biopsy as most of these connective tissue diseases are clinically diagnosed. Pertaining to your queries: 1. CENP B is quite a specific antibody for limited scleroderma. It co relates very well with your raynauds although raynauds itself can present along with Sjogrens syndrome. It is more like a symptom than a conclusion. But if CENP B is positive, it goes high in favor of limited systemic sclerosis. No, it is not common in healthy people 2. Yes, you can have raynauds for another few years before any symptoms of sclerosis develop Exact duration, one cant predict. But what you need to understand for the disease is that it is a very slow progressive. It can take up to 10-15 years before any further symptoms develop. Exact percentage one cant predict, but studies say 20-30 %. 3. As for CENP B positive, the most common problem is raynauds. Later patients develop skin tightening which is limited to fingers and feet. The next common thing you need to monitor is your Heart Pressures. Once a year 2d Echo to asses for pulmonary ARtery PRessure has to be done. Lungs and kidneys are less common of the involvement in CENP B positive patients 4. I doubt you would have sjogrens since childhood. Some event has happened in between which has triggered the antibody formation in you and caused these symptoms. HOpe the information has been useful DO let me know for more queries. Would be happy to help out Regards Dr Naval