Abnormal uterine bleeding (AUB) is a frequent gynaecological concern encountered in clinical practice and may stem from a broad spectrum of aetiologies ranging from structural abnormalities to systemic disorders. Among these, bleeding due to hematologic or autoimmune disorders, though less common, poses a significant diagnostic and therapeutic challenge. Autoimmune cytopenia’s and coagulopathies are rare but potentially life-threatening causes of bleeding and may occasionally be the first manifestation of a systemic autoimmune disease.

Sjogren’s syndrome, primarily known for its exocrine gland involvement causing dry eyes and mouth, may have significant extra-glandular manifestations including hematologic, renal, pulmonary, and neurologic involvement. In about 20% of cases, glandular symptoms may be absent, delaying diagnosis. Even more rarely, patients may initially present with haematological abnormalities like thrombocytopenia or acquired coagulopathies, including acquired factor inhibitors. This case highlights such an unusual presentation of primary Sjogren’s syndrome with abnormal uterine bleeding and purpuric rashes, ultimately diagnosed as a combination of immune thrombocytopenic purpura and acquired coagulation factor inhibitors due to lupus anticoagulant—without classical sicca symptoms.

A 30-year-old unmarried female, working as an accountant, presented to the outpatient department with a primary complaint of abnormal bleeding per Vaginum for 28 days with generalized fatigue and non-palpable purpuric rashes over her bilateral lower limbs, which appeared around 10 days before presentation. Her menstrual history prior to this episode was completely unremarkable—cycles had been regular, occurring every 28–30 days, with moderate flow lasting for 4–5 days with no prior episodes of intermenstrual bleeding or dysmenorrhea. However, in the current episode, she noticed unusual heavy bleeding from the onset, requiring frequent sanitary pad changes (every 4–5 hours) for the initial 5–6 days, which was followed by persistent low-grade bleeding over the next 20–25 days. The flow gradually reduced but did not completely cease.

She also reported fatigue that was present throughout the day, not relieved by rest, and worsened with routine activity. Concurrently, she developed non-tender, non-palpable red rashes over her lower limbs that were purpuric, non-blanching, non-palpable, measured 2 mm or more in diameter. She denied any history of trauma, drug intake (including NSAIDs, anticoagulants, hormonal agents), fever, oral ulcers, dry mouth or eyes, arthralgia, or recent travel. There was no past history of similar episodes, no known co-morbidities, and no family history of bleeding disorders. She followed a vegetarian diet, had normal bowel and bladder habits, and did not consume alcohol or tobacco.

On general physical examination, the patient was pale, afebrile, with a heart rate of 114/min, blood pressure of 114/66 mmHg, and oxygen saturation of 98% on room air. The conjunctiva and tongue appeared pale, consistent with anaemia. Petechial and purpuric rashes were noted over the lower limbs, non-blanching on diascopy, non-palpable, and non-tender. Systemic examination including neurological, gastrointestinal, and respiratory systems and cardiovascular was within normal limits. Gynaecological examination revealed an intact hymen and normal external genitalia. A per rectal examination confirmed a normal-sized, non-tender uterus with free parametrium, thus ruling out any gynaecological cause

Initial investigations included a complete blood count, coagulation profile, and peripheral smear. Laboratory results revealed a haemoglobin level of 7.8 gm/dL and a critically low platelet count of 15,000/µL. Prothrombin time (PT) and INR were within normal range; however, activated partial thromboplastin time (aPTT) was significantly prolonged at 72.6 seconds (control: 27 seconds). A urinalysis showed mild haematuria (persistent with the finding of per vaginal bleed), while other routine parameters including liver function tests, renal function tests, thyroid profile, ultrasonography of abdomen and pelvis (ruling out gynaecological cause), electrolytes were within normal limits.

In light of the low platelet count and active bleeding, she was transfused with one unit of single-donor platelets. However, post-transfusion, platelet counts did not show significant improvement. Before the transfusion, other investigations were sent, including a corrected reticulocyte count, iron profile, lactate dehydrogenase (LDH), serum Vitamin B12, Coombs test (direct and indirect), and stool for occult blood. Retic count was raised (4%) with negative coombs and stool occult blood report. Rest other reports were normal. Simultaneously, a coagulation workup was expanded to include fibrinogen levels (normal), immature platelet fraction, repeat aPTT (raised), and D-dimer (754 ng/ml).

Given the dermatological presentation, a dermatology consultation was sought. Skin lesions were assessed to rule out systemic vasculitis. Diascopy was negative, confirming purpura rather than vasculitic lesions. Based on clinical morphology and platelet levels, a working diagnosis of thrombocytopenia-related purpura was considered, possibly immune-mediated.

Further, given the isolated prolonged aPTT with a normal PT/INR, a differential diagnosis of either clotting factor deficiency or presence of an inhibitor was considered. A mixing study¹ was performed to differentiate these. The patient's plasma was mixed 1:1 with normal pooled plasma and aPTT was remeasured. The prolonged aPTT persisted after mixing, ruling out factor deficiency and strongly suggesting the presence of an inhibitor—either lupus anticoagulant (most common) or specific factor inhibitor. To differentiate between them, specific factor inhibitor assay (most common - factor 8) and antiphospholipid antibody panel was sent. Specific factor inhibitor assay report was normal but antiphospholipid antibody panel showed lupus anticoagulant positive, but negative anti-cardiolipin and anti-beta 2 glycoprotein antibodies.

To investigate an autoimmune etiology, an ANA test by immunofluorescence assay (IFA) was also performed and it turned to be positive with a speckled pattern (2++). Subsequently, an ANA profile revealed strong positivity for Anti-Ro60 and Anti-Ro52 antibodies (3+++), commonly associated with Sjogren’s syndrome. Despite the absence of classical sicca symptoms, a referral to ophthalmology was made to objectively assess glandular involvement. Schirmer’s test showed reduced tear secretion (<10mm), and tear film breakup time was recorded at 7–8 seconds in both eyes, indicating mild dry eye—supporting the diagnosis of primary Sjogren’s syndrome. Further, EULAR criteria^2,5 for Sjogren’s Syndrome (2016 criteria) was applied (Score - 4), which also confirmed presence of Sjogren syndrome

To assess clotting dynamics, a Thromboelastogram (TEG) was done which showed a hypocoagulable state characterized by prolonged reaction and kinetics time, low clot strength (MA), and low alpha angle—indicative of combined clotting factor deficiency and thrombocytopenia.

These results established a working diagnosis of an autoimmune-mediated bleeding disorder (Sjogren’s Syndrome), manifesting as immune thrombocytopenic purpura and acquired coagulation inhibitor syndrome (due to lupus anticoagulant).

Treatment was initiated promptly with pulse corticosteroids—intravenous methylprednisolone 1000 mg daily for 3 days—followed by oral prednisolone 30 mg twice daily for 6 weeks. Hydroxychloroquine 200 mg twice daily was added as a disease-modifying agent. Artificial tears were prescribed for mild dry eyes, and the patient was monitored closely for hematologic response and symptom resolution.

The patient was observed over 7 days of inpatient stay, with gradual clinical improvement: bleeding subsided, purpura resolved, platelet count improved, and her fatigue lessened. aPTT remained mildly prolonged (50 sec) but was expected due to persistent lupus anticoagulant. She was discharged on steroid regimen and advised close follow-up with repeat blood tests and ophthalmologic monitoring. After 6 weeks, steroids were gradually tapered

This case offers a striking example of how autoimmune disorders can present atypically and elude early diagnosis if classical symptoms are absent. Primary Sjogren’s syndrome (pSS)^3,4 is typically recognized by its glandular manifestations, specifically dry eyes and dry mouth resulting from lymphocytic infiltration and destruction of salivary and lacrimal glands. However, up to 20% of patients may present without these hallmark symptoms, instead manifesting with systemic, hematologic, or cutaneous features. Our patient had no sicca complaints but was eventually diagnosed with pSS based on immunologic markers (Anti-Ro60, Anti-Ro52, positive ANA), supported by ophthalmologic findings (reduced Schirmer’s and tear breakup time). This underscores the need for clinicians to maintain a high index of suspicion for SS in patients with unexplained systemic or hematologic findings.

• Autoimmune Cytopenia’s as an Initial Presentation

One of the most important insights from this case is the recognition of immune-mediated cytopenia’s as potential initial signs of systemic autoimmune disease. Immune thrombocytopenia (ITP) in the setting of pSS is a rare but documented phenomenon. It is believed to occur due to autoantibodies targeting platelet surface glycoproteins, leading to increased destruction and impaired production. The patient’s platelet count was critically low (15,000/μL) and unresponsive to transfusion—typical of ITP—and improved only after corticosteroid therapy, further supporting an autoimmune etiology.

The early and isolated presentation of ITP in pSS can mimic haematological malignancies or primary ITP, making diagnosis particularly difficult. Literature shows that hematologic abnormalities, such as anaemia, thrombocytopenia, leukopenia, and even acquired haemophilia, may precede the diagnosis of pSS by months or years. Hence, autoimmune evaluation, especially ANA and ENA panels, should be included early in the workup of unexplained cytopenia’s, particularly when standard therapies such as transfusions fail.

• Acquired Coagulation Disorders in Autoimmunity

In parallel to thrombocytopenia, our patient exhibited a markedly prolonged activated partial thromboplastin time (aPTT) with normal prothrombin time (PT), INR, and fibrinogen, suggesting an isolated defect in the intrinsic coagulation pathway. Differential diagnosis included clotting factor deficiencies (particularly factors VIII, IX, XI, XII), heparin exposure, and the presence of inhibitors such as lupus anticoagulant (LA) or specific factor inhibitors.

The mixing study was a pivotal test in this scenario. Persistence of prolonged aPTT despite mixing with normal plasma indicated the presence of an inhibitor rather than a simple deficiency. Further investigations confirmed the presence of lupus anticoagulant. This created a paradox, as lupus anticoagulant is commonly associated with thrombotic rather than haemorrhagic manifestations. However, bleeding can occur when lupus anticoagulant coexists with inhibitors to clotting factors, especially factor VIII, leading to a clinical picture resembling acquired haemophilia A.

This case thus highlights a rare coexistence of LA and acquired coagulation factor inhibitor, which has been scarcely reported. The simultaneous presence of two opposing coagulation pathologies (prothrombotic LA and haemorrhagic factor inhibitor) complicates the clinical picture and can result in diagnostic confusion unless carefully delineated through stepwise laboratory evaluation. Notably, the patient's Thromboelastogram (TEG) confirmed a hypocoagulable state, aligning with the clinical bleeding and laboratory findings.

• Sjogren’s Syndrome Without Sicca Symptoms

Sjogren’s syndrome, especially in its primary form, is often underdiagnosed due to its protean manifestations. When it presents without dryness, clinicians may fail to recognize it as a connective tissue disease. In our patient, the diagnosis was uncovered only after a detailed autoimmune panel and ophthalmologic assessment, confirming mild dry eyes through Schirmer’s and tear film breakup tests. According to the 2016 ACR/EULAR classification criteria, positive anti-Ro antibodies combined with ocular signs fulfil the diagnostic threshold even in the absence of symptomatic dryness.

This case reinforces the importance of objective testing (Schirmer’s, ANA, ENA) in suspected autoimmune disorders even when classical symptoms are missing. Multisystem presentations like haematological, dermatological (purpura), and gynaecological (abnormal bleeding) symptoms should prompt an autoimmune workup, especially in young females.

• Therapeutic Response and Management Principles

Our patient responded well to a combination of high-dose corticosteroids and hydroxychloroquine. Corticosteroids remain the first-line treatment for autoimmune cytopenia’s, as recommended by EULAR 2020 guidelines. Hydroxychloroquine is effective in controlling systemic features and is considered safe for long-term use. Platelet counts normalized, bleeding subsided, and purpuric rashes resolved, indicating both ITP and factor inhibitor-related bleeding were controlled.

For patients unresponsive to steroids or with severe bleeding, second-line agents such as rituximab, intravenous immunoglobulin (IVIG), or recombinant factor VIIa may be necessary. Our patient improved without the need for escalation, but she remains under observation for potential future flares or systemic complications such as interstitial lung disease or lymphoma, which are known long-term risks in pSS.

• Clinical Relevance and Diagnostic Pearls

This case exemplifies several important diagnostic principles:

• The evaluation of prolonged aPTT should always include a mixing study to differentiate between deficiency and inhibitor.
• Lupus anticoagulant, despite its name, may present with bleeding if combined with factor inhibitors.
• Objective assessment of dryness is critical in diagnosing SS without classical symptoms.

This case illustrates a rare presentation of primary Sjogren’s syndrome with hematologic manifestations—immune thrombocytopenia and acquired coagulation factor inhibitor (lupus anticoagulant)—without any glandular symptoms. The diagnosis was made based on a meticulous stepwise evaluation of prolonged aPTT, refractory thrombocytopenia, and autoimmune antibody profiling.

The presence of lupus anticoagulant typically correlates with thrombotic risk, yet this patient presented with bleeding—highlighting the paradoxical role of these antibodies when associated with clotting factor inhibitors. Sjogren’s syndrome should thus be considered in the differential diagnosis of unexplained cytopenia’s and coagulopathies, even in the absence of classic symptoms.

Uniqueness of the Case

• Absence of Classic Symptoms: The patient had no sicca symptoms, making this an atypical case of primary Sjogren’s syndrome.
• Initial Presentation as Cytopenia: The first sign was immune-mediated thrombocytopenia and bleeding, without other systemic symptoms.
• Simultaneous Lupus Anticoagulant and Factor Inhibitor: A rare coexistence of lupus anticoagulant with an acquired factor inhibitor resulted in both bleeding and an isolated prolonged aPTT.
• Multisystem Involvement Unfolding Over Time: The systemic autoimmune nature of the disease was revealed only through detailed and sequential testing.
• Diagnostic Challenge: The overlap of haematological and rheumatological features with absent classic symptoms required an astute diagnostic approach.

Take Home Message

• Always consider autoimmune aetiologies in patients with unexplained cytopenia’s and bleeding diatheses.
• Mixing studies are essential to distinguish factor deficiencies from inhibitors when evaluating prolonged aPTT.
• Presence of lupus anticoagulant can paradoxically present with bleeding when associated with factor inhibitors.
• Sjogren’s syndrome can manifest first with hematologic symptoms in the absence of sicca complaints.
• Hydroxychloroquine and corticosteroids remain the cornerstone of treatment; escalation to rituximab may be needed in refractory cases.
• In women of reproductive age, pregnancy-safe immunomodulation should be considered.

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