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

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 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

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 investigate the autoantibody profile and its clinical association in patients with systemic lupus erythematosus. METHODS: The frequency and clinical correlation of autoantibodies were studied in 73 patients with systemic lupus erythematosus who have been followed in Seoul National University Hospital. Double immunodiffusion, ELISA and immunoblot were used for the detection of autoantibodies. RESULTS: The frequency of each autoantibody measured by double immunodif fusion was as follows; anti-Ro 53.4%, anti-La 11.0%, anti-Sm 20.5%, anti-U1 RNP 20.5%. The frequency of each autoantibody by ELISA was as follows; anti-Ro 69.9%, anti-La 27.4%, anti-Sm 54.8%, anti-Ul RNP 68.5%, anti-dsDNA 72.6%, anti-cardiolipin 47.2% (IgG 43.1?0, igM 15. 3%). The frequency of each autoantibody by immunoblot was as follows; anti-Ro 15.1?0, anti-La 42. 5%, anti-Sm 46. 6%, anti-U1 RNP 42. 5%. anti-ribosomal P(P0) 27.4%. Anti-Ro was associated with decreased frequency of nephrotic syndrome. Anti-U1 RNP was associated with increased frequency of malar rash, Raynaud phenomenon and decreased frequency of nephritis. Patients with both anti-Ro and anti La had more frequent serositis than those with anti-l~o only. Patients with both anti-Sm and anti-U1 RNP had less frequent thrombocytopenia than those with anti-U1 RNP only. And patients with anti-Sm and anti-dsDNA had more frequent arthritis than those with only one of both antibodies. There was a positive correlation of autoantibody titers between anti-Ro and anti-La, anti-Sm and anti-U1 RNP, anti-dsDNA and anti-cardiolipin(IgG). Taking the result of immunoblot as a standard, both of double immunodiffusion and ELISA showed low sensitivity but high specficity for anti La. As for anti-Sm and anti-U1 RNP, double immunodiffusion showed low sensitivity but high specificity, whereas ELISA showed high sensitivity but low specificity. CONCLUSIONS: In our study, some autoantibodies (anti-Ro, anti-U1 RNP) were associated with certain clinical manifestations while others not. Immunoblot being used as a standard method, ELISA showed higher sensitivity but lower specificity for anti-La, anti-Sm and anti-U1 RNP compared with immunodiffusion. It is recommended that in interpretating the laboratory findings of these autoantibodies these parameters of each method should be considered.
Antibodies ; Arthritis ; Autoantibodies ; Blotting, Western* ; Enzyme-Linked Immunosorbent Assay* ; Exanthema ; Humans ; Immunodiffusion* ; Immunoglobulin M ; Lupus Erythematosus, Systemic ; Nephritis ; Nephrotic Syndrome ; Raynaud Disease ; Sensitivity and Specificity ; Seoul ; Serositis ; Thrombocytopenia

Autoinflammatory diseases (AIDs) refer to a broad range of genetically mediated conditions characterized by recurrent attacks of systemic inflammation with typical manifestations of fever, rash, serositis, lymphadenopathy, and musculoskeletal symptoms. The discovery of genetic basis for these conditions have led to the understanding of novel intracellular receptors for infectious and noninfectious danger signals in innate immunity. Innate immunity has a key role in the development of AIDs, in contrast with autoimmune disease, which arise from problems in adaptive immunity. Advances in understanding the molecular mechanisms of intracellular inflammatory cascades have led to renewed interest in its role in the pathogenesis of more common non-genetic autoinflammatory rheumatic conditions, such as Behcet's disease, gouty arthritis, Adult onset Still's diseases, and systemic onset juvenile arthritis. The characterization of cryopyrin (inflammasome) and its significant role in the activation of proinflammatory cytokines, such as IL-1beta and TNF-alpha in the development of AIDs, has provided rational targets of anti-cytokine biologic treatment for some of these conditions.
Adaptive Immunity ; Adult ; Arthritis, Gouty ; Arthritis, Juvenile ; Autoimmune Diseases ; Cytokines ; Exanthema ; Fever ; Humans ; Immunity, Innate ; Inflammation ; Lymphatic Diseases ; Serositis ; Tumor Necrosis Factor-alpha