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.