|Year : 2021 | Volume
| Issue : 2 | Page : 50-53
Secondary thrombocytopenia with primary Sjogren’s syndrome: A rare presentation
Sanchu T. K. Sreeraj, Trinayani Barua, Anupam Dutta, Sreemanta M Baruah, Balasubramanian B. V. Ramanan, Arijit Das
Department of Medicine, Assam Medical College and Hospital, Dibrugarh, India
|Date of Submission||23-Jul-2021|
|Date of Acceptance||27-Jul-2021|
|Date of Web Publication||05-Oct-2021|
Dr. Sanchu T. K. Sreeraj
PGT, Room No. 47, PG Hostel, Assam Medical College, Dibrugarh, Assam
Source of Support: None, Conflict of Interest: None
Primary Sjogren’s syndrome (pSS) is a chronic systemic autoimmune rheumatic disorder that is characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands. pSS is the most frequent connective tissue disorder after rheumatoid arthritis, affecting 0.3%–3% of the population. The prevalence of pSS is more common in women than men, with a sex ratio of 9:1. Occult pSS can rarely present as immune-mediated cytopenia. In this case report, we exemplify an atypical presentation of pSS that presented as thrombocytopenia. Hence, we should always consider pSS in the differential diagnosis of patients with unexplained cytopenias.
Keywords: Rituximab, sicca syndrome, Sjogren’s syndrome, steroids, thrombocytopenia
|How to cite this article:|
Sreeraj ST, Barua T, Dutta A, Baruah SM, Ramanan BB, Das A. Secondary thrombocytopenia with primary Sjogren’s syndrome: A rare presentation. Assam J Intern Med 2021;11:50-3
|How to cite this URL:|
Sreeraj ST, Barua T, Dutta A, Baruah SM, Ramanan BB, Das A. Secondary thrombocytopenia with primary Sjogren’s syndrome: A rare presentation. Assam J Intern Med [serial online] 2021 [cited 2021 Dec 3];11:50-3. Available from: http://www.ajimedicine.com/text.asp?2021/11/2/50/327550
| Introduction|| |
pSS is a systemic autoimmune disorder of an unknown etiology., It mainly affects exocrine organs, but it can also affect other organs.
In 1882, for the first time, the German Theodor Karl Gustav von Leber (1840–1917) described a dry inflammation of the ocular surface under the name of “keratitis filamentosa.”
In 1925, Henri Gougerot (1881–1955), a French dermatologist, was the first to describe that xerostomia and ocular dryness are part of a larger sicca syndrome resulting from dysfunction of the exocrine glands or their autonomic innervation.
Henrik Samuel Conrad Sjögren (1899–1986) was a Swedish ophthalmologist who described keratoconjunctivitissicca (KCS)—distinct from vitamin A deficiency xerophthalmia—using Rose Bengal and methylene blue staining techniques. He was the first person who explained the association between KCS and systemic disease beyond the field of ophthalmology.
In the early 1960s, the autoimmune origin of pSS was finally recognized. pSS causes highly varied clinical manifestations that may overshadow the sicca symptoms; the diagnosis may get delayed because of this.
The initial manifestations of pSS are reported to be tiredness, weight loss, peripheral neuropathy, proximal myopathy, interstitial lung disease, urolithiasis, and renal tubular acidosis. Common blood abnormalities are normocytic normochromic anemia, leukopenia, and thromocytopenia. Hematological abnormalities as presenting complaints have only been described rarely in individual case reports. Here, we report a patient with bleeding manifestations as an initial presentation.
| Case Report|| |
A 40-year-old male presented to the casualty with a history of passage of black tarry stool and blood in vomitus for one day. We admitted and treated him initially for upper gastrointestinal bleeding. However, the patient did not respond well. On evaluation, the patient also gave a history of bleeding gums, non-pruritic erythematous rashes over both legs for seven days. It was not associated with fever, myalgia, diarrhea, joint pain, and prior history of drug intake. His physical examination was remarkable, with bleeding spots in the gums and purpuric rashes over both legs; the patient had tachycardia and hypotension, was severely pale, and had subconjuctival hemorrhage. A fundoscopic examination was done, and it was normal. Systemic examination did not reveal any remarkable finding. Clinical suspicion of thrombocytopenia led to following blood investigations and the reports are as given in [Table 1].
Peripheral blood smear study
RBC series shows a microcytic hypochromic picture with moderate anisopoikilocytosis. A fair number of elliptocytes, ovalocytes, and tear drop cells were seen. The WBC series shows leukocytosis and neutrophilia with normal maturation. Platelets are markedly reduced.
Bone marrow aspiration study shows erythroid predominant hypercellular marrow with the presence of hypolobated megakaryocytes.
Direct and indirect Coombs test were negative.
HBsAg, anti-HCV, and HIV serology were negative.
Ultrasound abdomen was done, and it was normal.
Antinuclear antibody profile analysis revealed SSA/anti-Ro 60, SSA/anti-Ro 52, SSB/anti-La were positive.
Schirmers I test was positive, and there was no salivary gland enlargement.
Clinical and laboratory parameters were analyzed, and a diagnosis of pSS with secondary thrombocytopenia was made.
The patient was treated with IV fluids, proton pump inhibitors, and IV Methylprednisolone therapy for five days, but the patient did not improve. Then, we started intravenous immunoglobulin 2 g/kg for five days. Ten units of platelets and eight units of whole blood transfusion were given. The patient responded clinically but he persisted to have low platelet counts. Then, we gave anti-CD20 monoclonal antibody, Rituximab on day 1 and day 7. The patient clinically improved, was discharged, and was asked to come for a review after one week for the third dose of Rituximab. [Figure 1] shows an improved clinical status of the patient. During the review, the patient had no bleeding tendencies and repeat platelet count was increased to 28,000. The fourth dose of Rituximab was given, and the patient was asked to come for a review to the out patient department for further follow-up.
| Discussion|| |
pSS is a chronic inflammatory autoimmune disease, in which salivary and lacrimal gland functions are impaired because of lymphocytic infiltration. “Secondary Sjogren’s syndrome” (sSS) is associated with other connective tissue diseases (e.g., SLE and systemic sclerosis [SScl]) and autoimmune diseases (e.g., primary biliary cirrhosis, thyroiditis, and vasculitis).
The clinical manifestations of pSS can occur at diagnosis or during follow-up, even after more than 10 years, so careful monitoring of patients is needed. The manifestations due to lymphocytic infiltration around an epithelium of a target organ have a stable and indolent course in general (e.g., sicca syndrome, renal tubular acidosis, pulmonary involvement) whereas the autoimmune diseases linked to immune complexes or autoantibodies have a more unpredictable course, with flares and remissions.
Dry eye is a classic manifestation of pSS, and it affects more than 95% of patients. Patients can present with symptoms of to produce tear, foreign-body sensation, conjunctival inflammation, and eye fatigue. Keratoconjunctivitis sicca, blepharitis, bacterial keratitis, or corneal ulcer are the complications of ocular dry eye. Patients with dry mouth can present with soreness, adherence of food to the mucosa, dysphagia, and difficulties in speaking or eating, dental caries, tooth loss, periodontal involvement, lip dryness, and aphthous ulcer. Mycotic complications of dry mouth are oral candidiasis and angular cheilitis related to the loss of the antimicrobial action of saliva.
Dry eyes and dry mouth are collectively described as sicca symptoms. Sicca symptoms can have a substantial impact on a patient’s quality of life and currently, the treatment is symptomatic. Sicca symptoms as an initial complaint are seen only in 36% of patients. The time period taken for the onset of the first autoimmune symptom and sicca symptom averages around eight years.
In patients with pSS, hematological abnormalities are common. The most frequently encountered cytopenia is leukopenia, which is seen in around 14%–42% of patients with pSS. The prevalence of anemia is about 20% of pSS cases, which are usually normochromic normocytic. Thrombocytopenia is infrequent in pSS (5%–16% only). Even though the association is rare, screening for pSS should be done in those who present with features of immune thrombocytopenic purpura.
The presence of anti-Ro/SSA and/or anti-La/SSB autoantibodies is the other major factor in the diagnosis of pSS. Anti-Ro/SSA and/or anti-La/SSB autoantibodies are positive in 50%–90% and 25%–60% of patients with pSS, respectively.
Anti-Ro/SSA autoantibodies can be differentiated into two types: anti-Ro52 and anti-Ro60. There is no specific ANA fluorescence staining pattern for anti-Ro52/SSA and it is precipitin negative and is not detected by enzyme linked immunosorbent assays based on natural SSA/Ro. Patients with autoantibodies Ro52+ Ro60+ are likely to have pSS, whereas patients with Ro52+ Ro60− are not likely to have pSS.
In the absence of ocular symptoms, it may be hard to suspect pSS as a differential diagnosis of multisystem disease, hampering the diagnosis, which accounts for rare association of pSS and cytopenia.
It is, therefore, conceivable that a considerable number of patients with occult pSS may have a variable degree of cytopenias as first presentation. This should be well recognized, because pSS is a treatable condition and it also carries an increased risk of other associated diseases such as lymphoma.
| Conclusion|| |
The case just cited illustrates the association of thrombocytopenia and pSS, which requires a strong clinical suspicion in the early diagnosis and treatment of the patient.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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