Introduction Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome that is associated with a high mortality rate. HLH is characterized by hemophagocytosis and histiocytic proliferation (1). Primary HLH has a familial pattern and secondary HLH is reactive and also called macrophage activation syndrome (MAS). This type of HLH is usually acquired following autoimmune diseases, infection, and malignancy (2).The prevalence of HLH is not completely measurable because the diagnosis of this disease is hard and there are other comorbid diseases at the time of diagnosis. In a study that was done in Sweden from 1987 to 2006, the prevalence of HLH was 1.5 per million (3).The manifestations of HLH are fever and cytopenia. The diagnosis of HLH is made by clinical suspicion of physicians. The criteria for diagnosis of HLH include fever; splenomegaly; cytopenia (affecting at least two of three lineages in the peripheral blood); fasting triglyceride levels ≥3 mmol/L and/or fibrinogen level ≤1.5 g/L; serum ferritin level ≥500 ng/ml; CD25 level ≥2400 U/ml; decreased or absent natural killer (NK) cell activity; or hemophagocytosis in bone marrow, spleen, or lymph nodes (4).Adult-onset Still’s disease (AOSD) is an autoimmune disorder that can predispose patients to HLH. The co-incidence of AOSD and HLH is rare (5).In this report, we present a woman with a co-incidence of AOSD and HLH.

INTRODUCTIONGranulomatosis with polyangiitis (GPA) is a rare form of small vessel vasculitis characterized by multisystem necrotizing granulomatous lesions with different organs involvements such as the central nervous system, urinary tract, gastrointestinal tract, skin, and even eye (1-3). The main pathological characteristic of GPA is the positivity of autoantibodies against cytoplasmic components of neutrophil cells or C-ANCA as well as proteinase-3 (PR3) over-activation (4). Due to the multisystem involvement nature, the diagnosis of GPA is difficult. Additionally, GPA may overlap with other clinicopathological conditions such as infections, autoinflammatory disorders, connective tissue diseases, and even neoplasms (5,6). In this regard, the involvement of salivary glands is a very rare phenomenon. Herein, we described a case of GPA with submandibular salivary gland involvement followed by reviewing the literature on similar cases.