DISCUSSION
Sjögren’s is a chronic autoimmune disease associated with lymphocyte
hyper-reactivity and involvement of various body parts. It is
characterized by the infiltration of lymphocytes into glands that
produce saliva and tears. The occurrence of Sjögren’s varies from 0.1%
to 4.8% and is more common in women than men (female-to-male ratio of
9:1.3) .1 While gland involvement is typical, symptoms
can vary widely from no symptoms to frequent experiences of dry eyes and
mouth. Less common symptoms include non-Hodgkin’s lymphoma and kidney
inflammation. Therefore, even patients exhibiting typical Sjögren’s
symptoms may face diagnostic challenges 2,3.
The dilemma in diagnosing primary Sjögren’s often overlaps with
conditions like rheumatoid arthritis, systemic lupus erythematosus, and
cryoglobulinemia. Factors such as the presence of rheumatoid factor,
elevated circulating immunoglobulins, and autoantibodies to RO/SSA and
LA/SSB indicate activated lymphocytes.4 In our case,
the fact that RO/SSA was positive, along with a high ESR and a positive
Schirmer test, all point towards primary Sjögren’s. While ANA is an
important marker for autoimmune disorders, it is not very specific. To
confirm or rule out autoimmune conditions, we conducted an ENA
(Extractable Nuclear Antigens) panel which showed a positive result for
RO/SSA. RO/SSA is linked to both SLE and Sjögren’s, but in this
instance, since there were no typical photosensitive rashes or signs of
serositis and other consistent features, SLE was ruled out.5
An ESR above 50 mm/hr and anti-RO/SSA positivity strongly suggest extra
glandular involvement.6 Liver issues in patients with
primary Sjögren’s syndrome can be due to chronic viral infections or
autoimmune liver disease7. With high ALP levels in our
patient, we conducted serology for viruses and AMA, which came back
negative. LDH levels are often seen as a fatigue biomarker in primary
Sjögren’s syndrome8, and our patient had elevated LDH
levels as well. One of the factors associated with a higher incidence of
anemia is the presence of ANA, anti-RO, and anti-LA antibodies. A study
revealed that patients with primary Sjögren’s syndrome and anti-RO
antibodies have more systemic characteristics such as hematological
issues like anemia and thrombocytopenia. 9
Patients with primary Sjögren’s often experience hematological
manifestations, which are not uncommon. Unfortunately, these
manifestations receive less attention and are usually considered mere
laboratory abnormalities without clinical significance. Anemia is the
most common cytopenia affecting the majority of the patients with
Sjogren’s syndrome. Our patient showed similar lab results with a
hemoglobin count of 10.3 and a platelet count of 62,000, despite not
exhibiting the typical dryness symptoms of Sjögren’s.
Cytopenia can precede the typical dryness symptoms, making early
diagnosis challenging. Mild anemia in Sjögren’s is believed to result
from cytokine-mediated chronic inflammation, although the exact
mechanisms behind hemolysis and bone marrow suppression are still
unclear.
Reports suggest that anti-neutrophil and anti-RBC antibodies are found
in a significant percentage of patients. Iron metabolism disturbances
lead to anemia, while functional impairment of megakaryocytes results in
thrombocytopenia. While hematological manifestations are frequent in
various autoimmune diseases, they lack specificity for diagnostic
purposes on their own. Therefore, even in the absence of typical dryness
symptoms, hematological changes should not be overlooked in primary
Sjögren’s syndrome.
In conclusion, significant cytopenia could be the initial sign of
underlying primary Sjogren’s disease even without obvious symptoms.
Thus, primary Sjogren’s should be considered when investigating
unexplained cytopenia. Factors like being female, having a positive ENA
(Extractable Nuclear Antigens) panel result, and high ESR levels may
suggest primary Sjogren’s. Given the diverse presentations of primary
Sjogren’s, healthcare providers should remain attentive to less common
manifestations like hematological abnormalities.