Anyone positive for CN-1a autoantibody

ChookityPop

Senior Member
Messages
610
I wonder how many of you have been tested for this antibody?

I just tested positive snd waiting for the results on my muscle biopsy atm.
 

Zebra

Senior Member
Messages
1,113
Location
Northern California
Hi, @ChookityPop

I just responded to your Dysphagia thread, and wanted to pop over here to respond to your antibody question.

I have tested positive for this antibody ("moderately positive titer").

Neither a neuromuscular specialist nor a rheumatologist feel I fit the diagnostic criteria for IBM at the time (4 years ago).

However, the rheumatologist told me that she often sees this positive antibody in patients who go on to be diagnosed with Sjogren's Syndrome.

Have you been tested for anti-SSA and SSB antibodies, typically associated with Sjogren's Syndrome? These are also referred to as anti-Ro and anti-La.

Additionally:
"Anti-Ro/SSA antibody is the most prevalent MAA in myositis and frequently occurs together with anti-ARS antibodies or other MAAs." (from the Myositis Association)

Hope to hear from you.
 

ChookityPop

Senior Member
Messages
610
Hi Zebra, thank you for commenting!

Very interesting, can I ask if you have any muscle weakness or prematuer muscle fatigue? And if so, is it full body etc?

I have done so much reading on this since testing positive. I have severe muscle weakness / premature muscle fatigue in my whole body but what freaks me out is that I have IBM specific weakness in my thighs, fingers, forearms, dysphagia etc.

CN1a is seen in Sjogrens as you say as well as other autoimmune diseases. If I remember correct its seen in 5% of patients with Polymyositis or something along those lines too. And there is something called PM-Mito which I posted something on later down this comment. Im going to bed and just threw in some additional notes I have saved. There is a pretty significant amount of Sjogrens patients positive for CN1a indeed.



**Polymyositis with mitochondrial pathology or atypical form of sporadic inclusion body myositis: case series and review of the literature **

**Polymyositis with mitochondrial pathology (PM-Mito) is a rare form of idiopathic inflammatory myopathy with no definite diagnostic criteria and similarities to both PM and sporadic inclusion body myositis (s-IBM).** The aim of this study is to address the dilemma of whether PM-Mito is a subtype of inflammatory myopathy or represents a disease falling into the spectrum of s-IBM. Herein, **we report four female patients diagnosed with PM-Mito, highlighting their rather atypical clinical and histopathological characteristics that seem to indicate a diagnosis away from s-IBM. **

**Muscle weakness was rather proximal and symmetrical** and lacked the selective pattern observed in s-IBM. Patients had large-scale deletions in mtDNA, reflecting the mitochondrial component in the pathology of the disease.

Conclusively, our study adds to the limited data in the literature on whether PM-Mito is a distinct form of myositis or represents a prodromal stage of s-IBM. **Although the latter seems to be supported by a substantial body of evidence, there are, however, important differences, such as the different patterns of muscle weakness, and the good response to treatment observed in some patients. https://pubmed.ncbi.nlm.nih.gov/31055642/



Cytosolic 5'-nucleotidase IA (NT5C1A; cN-IA; cN1A; cN-1A; Mup44 antigen)

70
* NT5C1A protein: 44-kDa muscle antigen
* Methodology
* Western blot
* More sensitive (70%)
* Higher specificity: False positives 10%
* ELISA
* Sensitivity: Lower: 40% to 50%
* False positive results: More (Up to 30%)
* Frequency of NT5C1A antibodies
* IM-VAMP syndromes
* NT5C1A antibody titers: High in sIBM syndromes
* IM-VAMP
* sIBM
* IM-Mito
* Western blot methods
* sIBM: 51% to 75% sensitivity
* IBM-like syndromes with no vacuoles in muscle (IM-VAMP): 64%
* ELISA & Cell-based methods: 33% to 45% sensitivity 104
* Other immune myopathies
* DM & PM: 4% to 20%
* Systemic immune disorders: Increased frequency
* Sjögren syndrome: 20% to 37%
* Systemic lupus erythematosus: 14% to 20%
* Other controls: Few
* Normals: Rare
* HLA association: HLA-DRB1*03:01 & position 74 arginine

Infections
* HIV & HTLV viral infection: ? Increased frequency of sIBM
* HIV: Younger onset of sIBM, Mean age 55, Youngest 38 years 89
* Hepatitis C virus (HCV) antibodies: 28% 88
* No clinical differences between HCV antibody + & - patients

Variant syndromes
* Inflammatory myopathy with mitochondrial pathology
* Slower progression of weakness
* No vacuoles in muscle biopsy
* Autosomal dominant inheritance
* Similar clinical features to sporadic IBM
* Less or no: Inflammation: MHC-I upregulation by muscle fibers
* No HLA type association


Inflammatory Myopathy + Mitochondrial Pathology in muscle (IM-Mito), subtype of IM-VAMP 42
* Clinical
* Onset age: Mean 61 years; Range 43 to 71 years
* Weakness
* Quadriceps: Early & most severe (90%)
* Proximal
* Distal (40%): Wrist & finger flexion
* Face (50%)
* Symmetric (70%)
* Progression: Slow
* Overall: 1% to 4% per year
* Knee extension: 3% to 8% per year
* Slower than IBM
* Treatment
* Long term: No clear benefit
* Methotrexate
* Dose: 10 to 20 mg/week
* More effective: Patients with < 10% of fibers COX-
* Patients unresponsive to treatment may have IBM on repeat muscle biopsy
* Corticosteroid therapy: NO response
* Laboratory
* Serum CK: Normal or mildly elevated; Range 54 to 1200
*
* Muscle pathology
* Variant syndromes
* Inclusion body myositis
* IM-VAMP


https://neuromuscular.wustl.edu/antibody/infmyop.htm#mito
 
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