Autoimmune thyroiditis in the course of other autoimmune diseases such as systemic lupus erythematosus (SLE) is common because these disorders are attributed to the production of autoantibodies against various autoantigens. Beyond this association, autoimmune thyroiditis can occur before, during or after the development of SLE. In this report, we describe a female who presented with facial puffiness, lethargy and progressive abdominal distension. She was diagnosed with autoimmune thyroiditis followed by the diagnosis of SLE complicated by a massive ascites, a rare form of lupus peritonitis, which is sterile ascites that results from severe serositis. Her presentation was complex and posed a diagnostic challenge and dilemma to the physicians involved in her care.
Thyroiditis, Autoimmune ; Lupus Erythematosus, Systemic ; Serositis

Autoimmune thyroiditis in the course of other autoimmune diseases such as systemic lupus erythematosus (SLE) is common because these disorders are attributed to the production of autoantibodies against various autoantigens. Beyond this association, autoimmune thyroiditis can occur before, during or after the development of SLE. In this report, we describe a female who presented with facial puffiness, lethargy and progressive abdominal distension. She was diagnosed with autoimmune thyroiditis followed by the diagnosis of SLE complicated by a massive ascites, a rare form of lupus peritonitis, which is sterile ascites that results from severe serositis. Her presentation was complex and posed a diagnostic challenge and dilemma to the physicians involved in her care.
Thyroiditis, Autoimmune ; Lupus Erythematosus, Systemic ; Serositis

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease of unknown etiology and is characterized by presence of variable pathogenic auto-antibodies and multiple organ involvement. Serositis is common in SLE, but peritoneal involvement is relatively rare. This is a case report of 28-year-old female who initially presented with abdominal pain and ascites. After ruling out many other possibilities such as liver cirrhosis, neoplasm, and infectious etiologies, we confirmed SLE with clinical features, serologic tests and radiological findings. To conclude, her abdominal pain and ascites were caused by lupus peritonitis. After administration of corticosteroid therapy, her symptoms fairly improved.
Abdominal Pain ; Adult ; Ascites* ; Female ; Humans ; Liver Cirrhosis ; Lupus Erythematosus, Systemic* ; Peritonitis ; Serologic Tests ; Serositis

Cytophagic histiocytic panniculitis gap (CHP) was described in 1980 as a chronic histiocytic disease of the subcutaneous tissue and associated with systemic manifestations such as fever, serositis, and hepatosplenomegaly. The current concept of CHP is that it represents a spectrum of lymphoproliferative disorders that induce secondary histiocytic cytophagocytosis. The pathogenesis of hemophagocytosis in CHP is unknown but may be related to histiocyte reaction to an abnormal cytokine milieu brought about by disordered T-cell function. In most cases the disease follows a fulminant course, but in some patients the disease seems limited to the skin and subcutaneous tissue and follows a more benign, chronic course. We report a case of cytophagic histiocytic panniculitis which had a long chronic course over 16 years, but recently became aggravated with the involvement of the extrapleural fat tissue and was treated with combination chemotherapy.
Cytophagocytosis ; Drug Therapy, Combination ; Fever ; Histiocytes ; Humans ; Lymphoproliferative Disorders ; Panniculitis* ; Serositis ; Skin ; Subcutaneous Tissue ; T-Lymphocytes

OBJECTIVE: To investigate the clinical and immunological characteristics of systemic lupus erythematosus (SLE) with retinopathy. METHODS: Fifty SLE patients with retinopathy without hypertension and diabetes (retinopathy group) who were hospitalized in the Peking University People's Hospital from January 2009 to July 2022 were screened. Fifty SLE patients without blurred vision during the course of the SLE and without retinopathy in the fundus examinations (non-retinopathy group) matched for sex and age were selected. Their clinical manifestations, laboratory tests and lymphocyte subsets were statistically analyzed. RESULTS: The most common fundus ocular change of the SLE patients with retinopathy was cotton-wool spots (33/50, 66.0%), followed by intraretinal hemorrhage (31/50, 62.0%). Retinopathy could occur at any stage of SLE duration, with a median of 1 year (20 days to 30 years). The proportion of lupus nephritis (72.0% vs. 46.0%, P=0.008) and serositis (58.0% vs. 28.0%, P=0.002) in the retinopathy group were significantly higher than those in the non-retinopathy group. The proportion of neuropsychiatric systemic lupus erythematosus (NPSLE) in the retinopathy group was higher, but there was no significant difference between the two groups. Compared with the non-retinopathy group, the proportion of positive anti-cardiolipin antibody (30.0% vs. 12.0%, P=0.027), the SLEDAI score (median 22.0 vs. 10.5, P < 0.001), erythrocyte sedimentation rate (P < 0.001), C-reactive protein (P=0.019) and twenty-four hours urine total protein level (P=0.026) in the retinopathy group were significantly higher, and the hemoglobin level was significantly lower [(91.64±25.18) g/L vs. (113.96±18.57) g/L, P < 0.001]. The proportion of CD19⁺ B cells in peripheral blood of the patients with SLE retinopathy was significantly increased (P=0.010), the proportion of CD4⁺ T cells was significantly decreased (P=0.025) and the proportion of natural killer (NK) cells was lower (P=0.051) when compared with the non-retinopathy group. CONCLUSION Retinopathy in SLE suggests a higher activity of SLE disease with more frequent hematologic and retinal involvement. It is recommended to perform fundus examination as soon as a patient is diagnosed with SLE. SLE patients with retinopathy may have stronger abnormal proliferation of B cells, and aggressive treatment should be applied to prevent other important organs involvement.
Humans ; Lupus Erythematosus, Systemic ; Lupus Vasculitis, Central Nervous System ; Lupus Nephritis ; Antibodies, Anticardiolipin ; Serositis

Adult-onset Still's diseas e(AOSD) is a rare, systemic inflammatory disease of unknown etiology, characterized by spiking fever, skin rash, arthralgia or arthritis, lymphadenopathy, hepatosplenomegaly, sore throat and serositis. The typical skin rash in AOSD is an evanescent, erythematous maculopapular lesion, often associated with spiking fever. However, other atypical skin lesions such as urticaria are rarely seen. Here, we report a case of a 24-year-old female patient in whom an urticaria-like rash was an uncommon clinical feature of AOSD.
Arthralgia ; Arthritis ; Exanthema ; Female ; Fever ; Humans ; Lymphatic Diseases ; Pharyngitis ; Serositis ; Skin ; Still's Disease, Adult-Onset* ; Urticaria* ; Young Adult

Familial Mediterranean fever (FMF) is the most common Mendelian autoinflammatory disease, characterized by uncontrolled activation of the innate immune system that manifests as recurrent brief fever and polyserositis (e.g., peritonitis, pleuritic, and arthritis). FMF is caused by autosomal recessive mutations of the Mediterranean fever gene, MEFV which encodes the pyrin protein. Although FMF predominantly affects people from Mediterranean and Middle Eastern ethnic origins, 3 cases of FMF have been reported in Korea since 2012. We report another case of FMF in Korea in which the patient presented with a month-long fever without serositis. After treatment with colchicine was initiated, the patient’s symptoms quickly subsided. The response to colchicine was helpful for diagnosis. We compare the FMF genotypes in Korea with in other countries. Studying FMF cases in Korea will help establish the best MEFV exons to use for screening and diagnosis of Korean FMF.
Colchicine ; Diagnosis ; Exons ; Familial Mediterranean Fever* ; Fever of Unknown Origin* ; Fever* ; Genotype ; Humans ; Immune System ; Korea* ; Mass Screening ; Peritonitis ; Serositis

Adult onset Still's disease (AOSD) is an uncommon acute systemic inflammatory disease of unknown origin. The clinical features include high spiking fever, arthralgia or arthritis, transient maculopapular rash, lymphadenopathy, hepatosplenomegaly, and serositis. The laboratory findings include leukocytosis(neutrophilia), elevation of AST, ALT, ESR, CRP and serum ferritin, and negative rheumatic factor and anti-nuclear antibody. We report a case of a 52-year old woman whom symptom is subsided after steroid use.
Adult* ; Arthralgia ; Arthritis ; Exanthema ; Female ; Ferritins ; Fever ; Humans ; Leukocytosis ; Lymphatic Diseases ; Middle Aged ; Serositis ; Still's Disease, Adult-Onset*

OBJECTIVE: To understand the clinical manifestations and disease course of adult onset Still' s disease (AOSD). METHODS: 15 patients of AOSD diagnosed at Severance hospital, Yonsei University College of Medicine were retrospectively analysed in the period of September 1988 to September 1995. RESULTS: There were 3 men and 12 women (male to female ratio of 1:4). Age of disease onset ranged from 17-55 years, and over 86% of the patients were younger than age 40 at disease onset. The prevalence of clinical features were as follows fever (100%), arthritis (93%), skin rash (93%), sore throat (60%), abnormal liver function (73%), lymphadenopathy (47%), splenomegaly (47%), hepatomegaly (20%), serositis (13%). Fever was the most common initial symptom. Common labaratory features were leukocytosis with neutrophilia (87%), anemia' Hgb <10 g/dL (67%), increased serum ferritin (83%), ESR (87%) and CRP (93%). Serum ferritin was markedly raised at disease onset and correlated with disease activity. In 2 patients, the disease was controlled with NSAID alone, but most of the patients required steroid to control the disease activity. In 6 patients, MTX was added for steroid sparing effect and for steroid resistant arthritis. Most of AOSD patients had intermittent and chronic disease course. Root joint arthritis and polyarthritis were factors associated with chronicity. CONCLUSION: The clinical features of AOSD in our study generally resemble previous reports. Serum ferritin was a useful marker of disease activity. Most patients of AOSD had intermittent and chronic disease course. Root joint athritis and polyarticular pattern at disease onset were factors associated with chronicity.
Adult* ; Arthritis ; Chronic Disease ; Exanthema ; Female ; Ferritins ; Fever ; Hepatomegaly ; Humans ; Joints ; Leukocytosis ; Liver ; Lymphatic Diseases ; Male ; Pharyngitis ; Prevalence ; Retrospective Studies ; Serositis ; Splenomegaly ; Still's Disease, Adult-Onset*

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin's lymphoma (NHL) and that progresses rapidly and is usually fatal. Because it usually presents with nonspecific symptoms, such as fever, the early diagnosis of IVLBCL is very difficult and it is often misdiagnosed as another disease. Systemic lupus erythematosus (SLE) is an autoimmune disease that affects various organs. The clinical manifestation of SLE ranges from rash and arthritis through anemia and thrombocytopenia to serositis, nephritis, seizures, and psychosis. Thus, it can be easily confused with many other disorders. We report a case of IVLBCL mimicking SLE in the initial diagnosis.
Anemia ; Arthritis ; Autoimmune Diseases ; B-Lymphocytes* ; Diagnosis ; Early Diagnosis ; Exanthema ; Fever ; Lupus Erythematosus, Systemic* ; Lymphoma ; Lymphoma, B-Cell* ; Lymphoma, Non-Hodgkin ; Nephritis ; Psychotic Disorders ; Seizures ; Serositis ; Thrombocytopenia