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This case details a 33-year-old female who presented with a suggestive autoimmune history, arthralgias, and splenomegaly, and tested positive for Sjögren’s syndrome and anti-liver–kidney microsomal antibody. This was further validated by findings from a liver biopsy, confirming a very rare association with Type 2 autoimmune hepatitis. Primary Sjögren’s syndrome is a sporadic disease with a global prevalence of 61 per 100,000 people and a total prevalence of 0.4% for secondary Sjögren’s syndrome. The prevalence of autoimmune hepatitis in association with primary Sjögren’s syndrome is 4–47%. It is divided into two types, associated with characteristic antibodies. Type 2 autoimmune hepatitis is rarely reported with Sjögren’s syndrome; much of the association reported in the literature has been with Type 1 autoimmune hepatitis.

Haemophagocytic lymphohistiocytosis (HLH) is a rare but potentially aggressive and life-threatening syndrome of overactive histiocytes and lymphocytes that commonly affects infants; it is also observed in children and adults of all ages. The disease is differentiated into either primary or secondary causes. Primary HLH tends to be of genetic origin, while secondary HLH results from either infection, autoimmune disorders, or malignancies. Secondary HLH is most commonly associated with viral infections in immunocompromised patients. This paper presents a case of HLH in a tertiary care hospital, associated with adult-onset Still’s disease, diagnosed on both biochemical criteria and histopathologic examination of bone marrow smear.

A 45-year-old Asian woman was presented with fever, easy fatigability, shortness of breath, cervical and axillary lymphadenopathy and other signs and symptoms of anemia. After all the baseline work-up, the patient was investigated for Mono-coombs C3d levels, which were elevated, suggesting the diagnosis of Cold autoimmune hemolytic anemia (Cold AIHA). An Ultrasound-guided true-cut biopsy was done to determine the primary cause associated with it, which showed the presence of tumor cells arranged in cords and clusters. They have dark staining cells with mitotic activity, suggestive of breast carcinoma as an association of Cold AIHA. Estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER2) were sent, which came out to be positive. So, the patient was diagnosed with Cold AIHA in association with triple-positive breast cancer.

Lupus panniculitis occurs in 1–3% of the patients diagnosed with systemic lupus erythematosus (SLE) and 10% of the patients diagnosed with discoid lupus erythematosus (DLE). It is a disorder of autoimmune origin, manifesting as deep erythematous plaques and nodules involving the trunk, breasts, buttocks, face, and proximal extremities. It does not commonly ulcerate. This report highlights the case of a 22-year-old Asian female with a history of coeliac disease and significant family history of antiphospholipid antibody syndrome (APS) who presented with fever, malaise, weight loss, and subcutaneous non-tender nodules over the forearm, back, bilateral thighs, and feet. Laboratory investigations revealed positive antinuclear antibodies, anti-Ro/SSA antibody, and lupus anticoagulant, resulting in a diagnosis of APS. Biopsies of lesions were consistent with findings of lupus panniculitis. Every case of SLE and DLE with discrete skin lesions should be reviewed for any distinct entity such as lupus panniculitis, as it may be associated with greater risk of flares and systemic involvement. The purpose of this case report is to emphasise that early diagnosis and prompt treatment is crucial to improving the prognosis of such patients.