UNASSIGNED: Antinuclear antibody-negative full-house lupus nephritis though previously reported, is fairly uncommon. Some patients go on to develop antibodies later in the disease course. The presence of RO-52 antibody in this case suggests an underlying immunological cause. Swift management based on strong clinical suspicion can be life-saving to the patient.
UNASSIGNED: Lupus nephritis (LN) is a serious complication of systemic lupus erythematosus (SLE) and is more likely to progress to end-stage renal disease (ESRD). With the recent EULAR/ACR criteria mandating antinuclear antibody (ANA) positivity as an entry criterion, clinicians are faced with a diagnostic dilemma in diagnosing cases of seronegative SLE. We present the case of a 25-year-old female who presented with photosensitive malar rash, hair loss, oral ulcers, menorrhagia, and kidney dysfunction, suggestive of SLE. Her ANA tests were negative, raising doubts about the diagnosis. Biopsy was delayed owing to anemia and thrombocytopenia, and clinical judgment led to the patient being diagnosed with LN, with prompt treatment resulting in significant improvement. Renal biopsy subsequently confirmed the case as diffuse class IV LN with full-house nephropathy. This case highlights the limitations of relying solely on ANA positivity in diagnosing LN and underscores the need for a comprehensive diagnostic approach for SLE that incorporates clinical features, immunological markers, and patient demographics. ANA-negative SLE patients demand heightened clinical suspicion, especially when other diagnostic parameters align with the disease. Swift intervention with immunosuppressive therapy, as seen in this case, can be life-saving.

UNASSIGNED: IgG4-related disease is a rare and emerging pathology, characterized by the appearance of pseudotumors. Due to the ability to mimic other pathologies, it is essential to consider it as a differential diagnosis in multisystemic processes. The diagnosis is challenging, requiring a multidisciplinary approach, to minimize the associated morbidity and mortality.
UNASSIGNED: IgG4-related disease (IgG4-RD) is a rare, emerging, systemic and chronic pathology, characterized by the appearance of pseudotumors resulting from tissue infiltration by IgG4-positive plasma cells that promote eosinophilic inflammation of the tissue with subsequent fibrosis. We present the case of a male, 45-year-old patient, with marked weight loss and skin pallor detected by his family doctor during a child health consultation of his daughter. When questioned, the patient referred complaints of postprandial discomfort in the left hypochondrium with a feeling of fullness, weight loss, chronic fatigue and hyperhidrosis that had lasted for a month. On physical examination, he was pale, and had pain at palpation of the left hypochondrium. Laboratory data showed increased inflammation markers, abdominal ultrasound and CT demonstrated numerous enlarged lymph nodes in the upper quadrants, raising concern for a malignant lymphoproliferative process. Serological, imaging, clinical and laparoscopic excisional biopsy revealed features of IgG4-related disease and excluded malignant lymphoproliferative disease. The immediate response to treatment with oral prednisolone 30 mg/day also contributed for diagnosis confirmation. Due to refractory disease after gradual prednisolone reduction, second-line therapy with rituximab was initiated. Over the 6 years of follow-up, the patient presented multiple exacerbations characterized by the emergence of systemic symptoms, being maintained under close clinical and imaging follow-up by reumathology, infectious diseases, and family medicine specialists.

UNASSIGNED: Mixed connective tissue disease (MCTD) is characterized by high titres of distinct antibodies: U1 ribonucleoprotein with variable clinical features seen in rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis, and dermatomyositis. Limited case reports revealed the association between MCTD and cancer, like lymphoma, lung cancers, and others.
UNASSIGNED: A 22-year-old female presented with enlargement of the abdomen and oedema of the lower extremities, gradually started 25 days The patient had been diagnosed to have rheumatoid arthritis. She was treated with 7.5 mg/week MTX for 6 months. Physical examination revealed: pallor, lower limb oedema, with synovitis and deformities of hands. The laboratory tests showed anaemia, elevated levels of creatine phosphokinase ESR, positivity of antinuclear antibody, anti-ds DNA, and antinuclear ribonucleoprotein. Urinary protein excretion was 1625 mg/24 h. Chest X-ray showed bilateral pleural effusion. Echocardiography revealed pericardial effusion Thoracic-abdominal and pelvic tomography showed a heterogeneous mass with a diameter of 5 × 6 cm at the expense of the right ovary. The mass was removed surgically, and a biopsy was taken, and was compatible with ovarian high-grade serous adenocarcinoma. A course of solumedrol 1 g/IV/3 days was applied, and then continue with 60 mg/day oral predlone. Later on discharge, she was taken 25 mg/day predlone, and methotrexate 10 mg.
UNASSIGNED: Our case showed that the patient had no risk factors for developing ovary cancer. On the contrary, our patient was a young, non-smoker, without any previous treatment before the RA diagnosis was taken, and finally, she had 3 children with full-term pregnancy, and well health. This case highlights the importance of maintaining a high index of suspicion for malignancy in MCTD patients. However, further investigation on the role of the immune system in the development of ovarian cancer in women with autoimmune diseases including MCTD remains necessary.

A rare association exists between systemic lupus erythematosus (SLE) and multiple myeloma (MM). SLE is associated with a variety of symptoms. A combination of MM and SLE is uncommon in the young population. An unusual case of SLE associated with MM is described here. We present the case of a 39-year-old woman who was a known case of SLE and presented with severe chest and abdominal pain. We summarize the clinical characteristics of MM in SLE. The possible mechanisms that could be at the root of this association are also discussed.

Systemic amyloid light chain, or primary amyloidosis (AL amyloidosis), is a serious medical condition that leads to the deposition of abnormal proteins called amyloid fibrils in various organs of the body. AL amyloidosis can present with different symptoms, which can make diagnosis challenging. This case report presents a clinical scenario of a 53-year-old female patient who had come in for shortness of breath and lower extremity swelling and was found to have acute on chronic pulmonary embolism. The patient had a history of systemic amyloidosis diagnosed with a kidney and duodenal biopsy. She also had a bone marrow biopsy done and was found to have IgG monoclonal gammopathy. Throughout the hospital course, patients required cautious diuretic use given the worsening kidney function. She was given intravenous anticoagulation initially and later switched to oral medication on discharge. Due to the aggressive nature of amyloidosis, a decision was made to start the patient on chemotherapy in an outpatient setting. This case presents an interesting scenario of systemic amyloidosis with concomitant monoclonal gammopathy that was complicated by acute pulmonary embolism. The case is important as it shows the different levels of amyloidosis and teaches us the benefit of taking a multidisciplinary approach to making a concrete plan for patients with advanced amyloidosis disease.

BACKGROUND: Intrapancreatic accessory spleen (IPAS) is a congenital entity that can be confused with malignant distal pancreatic masses. Radiologic imaging and radionuclide imaging have an important place in the diagnosis of IPAS.
METHODS: Blood tests were performed on a 36-year-old female patient who presented with tachypnea, tachycardia, pain in the joints, and pain in the left abdominal quadrant. Laboratory test results were as follows: hemoglobin value 6.0 mg/dl, sedimentation 120, aspartate transaminase (AST) 150U/L, and alanine transaminase level (ALT) 110U/ L. Additional laboratory tests and ultrasonography were performed. The anti-double-stranded DNA (dsDNA) level was 800 IU/ml. C3 and C4 values were both 0.64 IU/ml, with anti-Ro-52 +++(three positive) and anti-Ro-60 ++ (two positive). A clinical diagnosis of systemic lupus erythematosus (SLE) was made. Ultrasonography and dynamic contrast-enhanced upper abdominal MRI showed lesions suggestive of multiple hemangiomas in the liver and a 29x18 mm lesion in the tail of the pancreas with a similar appearance as the spleen. SLE treatment was started. Scintigraphy was recommended for the diagnosis of IPAS. Scintigraphy was performed in the third week of the treatment. Uptake was not observed. In the second month of the treatment, a control upper abdominal MRI was performed, and a decrease in the size of the lesion was observed.
CONCLUSIONS: IPAS can be confused with pancreatic masses. Lack of uptake in scintigraphy may be due to treatment protocols that produce low phagocytic activity. If radiological imaging findings are compatible, a reduction in lesion size after immunosuppressive therapy can be accepted as evidence for the diagnosis of IPAS.

Leukocytoclastic vasculitis, also known as hypersensitivity angiitis, is a cutaneous, small vessel vasculitis of the dermal capillaries and venules. The predominant clinical presentation is palpable purpura. Multiple medications can cause leukocytoclastic vasculitis, as well as autoimmune diseases, infections, and malignancy. The disease process may be limited to only the skin or a manifestation of a systemic vasculitis or process. Treatment is centered on symptom management. Our patient is a 60-year-old female who presented with bilateral dry and wet tender ulcerations. She was previously treated with paclizumab.

UNASSIGNED: To explore the lessons learned from the misdiagnosis of systemic lupus erythematosus (SLE) combined with urinary tuberculosis leading to tuberculous meningitis (TBM) and the diagnosis and treatment of TBM through case reports and review of the literature.
UNASSIGNED: We report a case of an SLE patient presenting with urinary tuberculosis infection misdiagnosed as interstitial cystitis and complex urinary tract infection, who developed neurological infection after a cystocentesis biopsy and was eventually diagnosed with TBM. In addition, all cases of SLE combined with TBM from January 1975 to February 2022 were summarised and reviewed to compare current diagnostic and treatment strategies for the disease.
UNASSIGNED: The patient suddenly developed neurological symptoms after cystocentesis biopsy, and we detected Mycobacterium tuberculosis in the macrogenomic next-generation sequence (mNGS) of the cerebrospinal fluid. We therefore excluded interstitial cystitis and neuropsychiatric lupus to confirm the diagnosis of Mycobacterium tuberculosis infection leading to urinary tract tuberculosis and TBM.
UNASSIGNED: SLE is complicated by urological tuberculosis, surgery triggering hematogenous dissemination leading to tuberculous meningitis. At the same time, the lack of specificity in the clinical presentation of patients makes it easy to misdiagnose neuropsychiatric lupus and delay treatment, so timely and accurate diagnosis and effective anti-tuberculosis treatment are essential.

Ischemic vaso-occlusive retinopathy as an initial manifestation is rare in pediatric systemic lupus erythematosus (pSLE). A 13-year-old girl presented with two months\' history of papules and crusts with fatigue, weight loss, and abrupt hair loss. Pancytopenia and findings compatible with SLE, including positive direct Coombs\' test, antinuclear antibody (Ab), anti-double stranded DNA Ab, anti-Smith Ab, anti-ribonucleoprotein Ab, lupus anticoagulant, anti-β2 glycoprotein Immunoglobulin G, and anti-cardiolipin Ab, were detected. Bi-nasal hemianopsia was detected. Initial visual acuity was hand motion in the right eye and 15/20 in the left. Fundoscopy showed massive exudation around the optic disc with macular edema, vascular sheathing with perivascular hemorrhage in the whole retina, and ghost vessels in the peripheral retina. Intravitreal triamcinolone injection and dexamethasone implant injection were administered. Visual symptoms improved but did not recover. Methylprednisolone therapy and photocoagulation improved visual acuity and fever. Early intervention for retinopathy in pSLE can help prevent vision-loss.

Mixed cryoglobulinemic vasculitis (CV) is occasionally caused by autoimmune diseases including systemic sclerosis. Multiorgan involvement such as skin, kidney, and peripheral nerve involvement is common in mixed CV. However, central nervous system (CNS) involvement is extremely rare. Here, we report a case of overlap syndrome of limited cutaneous systemic sclerosis and mixed cryoglobulinemic vasculitis with CNS involvement. The neurologic deficits and systemic symptoms improved promptly after steroid and cyclophosphamide therapy.