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ABSTRACT This case highlights the importance of taking Systemic Lupus Erythematosus seriously and its possible concurrent musculoskeletal complications, as they can have significant health implications. Consulting and encouraging patients to adhere to the treatment is crucial. Although this case survived osteomyelitis without amputation, this outcome is rare and cannot be generalized.

The deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive disorder caused by loss of function mutations in the ADA2 gene (previously the CECR1 gene) on chromosome 22q11. The clinical spectrum of the disease is remarkably broad, and its presentations mimic features of polyarteritis nodosa, such as livedoid rash, hematological abnormalities (e.g., cytopenia), early‐onset stroke, hypogammaglobulinemia, and systemic inflammation. Early diagnosis and treatment of DADA2 are crucial, as the clinical features could be potentially life‐threatening but treatable. In this study, a 17‐year‐old male patient is reported with DADA2 whose symptoms mimic those of polyarteritis nodosa. A 17‐year‐old male patient presented with a 14‐year history of abdominal pain, hypertension, and cutaneous lesions initially attributed to polyarteritis nodosa (PAN). He was referred to our center due to ongoing abdominal pain. An abdominal and pelvic computed tomography scan with contrast revealed a retroperitoneal hemorrhage compressing the left kidney. Given his history of abdominal pain, hypertension, hemiparesis, transient ischemic attacks (TIA), anemia, cutaneous lesions, and retroperitoneal hemorrhage, DADA2 was suspected, and a genetic test confirmed the diagnosis. Treatment with anti‐TNF (Adalimumab) was initiated, resulting in noticeable improvement. In the follow up, fever, abdominal pain and TIA episodes were subsided and now he has a good clinical condition. Considering DADA2 and conducting a broad screening for other manifestations is recommended for patients presenting with PAN‐like symptoms. These patients may become symptomatic later in life, and early diagnosis allows for the consideration of disease‐specific treatment options. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Malignancy may be a possible cause of systemic lupus erythematosus (SLE) flare‐ups, and it is necessary to consider it in the context of treatment resistance. In this case, we present a challenging instance of concomitant nodal marginal zone B‐cell lymphoma (NMZL) and SLE flare‐up in a 41‐year‐old male patient. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can cause various symptoms and affect multiple organs in the body. It is also associated with the development of malignancies, especially lymphomas. This case report discusses a patient who experienced a flare‐up of SLE along with hypercalcemia, which led to the diagnosis of nodal marginal zone B‐cell lymphoma (NMZL). This is the first case of its kind to be reported. A 41‐year‐old man with a 10‐year history of SLE and antiphospholipid syndrome (APS) was referred to our center due to several symptoms, including fatigue, oral lesions, dyspnea, bilateral wrist pain and inflammation, mild pericardial effusion, organ enlargement, pancytopenia, high erythrocyte sedimentation (ESR) level, high anti‐double stranded DNA (anti‐dsDNA) level, low complement level, resistant hypercalcemia, and high brain natriuretic peptide (pro‐BNP) level. After further testing, it was discovered that the patient had NMZL, which was the ultimate diagnosis. He underwent six cycles of the R‐CHOP chemotherapy regimen, and his clinical and laboratory conditions improved during follow‐ups. The initial case of SLE flare‐up, with concomitant NMZL is being reported as the final diagnosis. In simpler terms, it is possible for lymphoma to manifest as a potential cause of SLE flare‐ups, and clinicians should be mindful that they need to consider malignant conditions when faced with treatment resistance. [ABSTRACT FROM AUTHOR]

Background: Dermatomyositis (DM) is a systemic autoimmune disease characterized by distinct skin lesions and a clinically heterogeneous constellation of systemic manifestations. This disease poses a challenge to clinicians because of its rarity, diverse clinical presentations, and variable organ involvement, resulting from an autoimmune attack on affected organs, which could be triggered by environmental factors in genetically susceptible individuals. Renal involvement is rare, with immunoglobulin M (IgM) nephropathy yet to be reported in patients with DM. Case presentation: A 38‐year‐old man was admitted to Shariati Hospital, affiliated with Tehran University of Medical Sciences, with proximal weakness of the upper and lower extremities that had developed in the preceding month after receiving the Sinopharm COVID‐19 vaccine. The patient was diagnosed with DM based on the heliotrope rash, Gottron's papules, progressive proximal muscle weakness, and paraclinical findings. IgM nephropathy developed subsequently, diagnosed by light and immunofluorescence microscopy. Conclusion: We describe the first case of IgM nephropathy in a DM patient following COVID‐19 vaccination. This phenomenon requires further investigation into the possible crosslinks between the pathogenesis of IgM nephropathy with DM and the COVID‐19 vaccine. Diagnosing renal complications in DM patients promptly and accurately can help to achieve the best outcomes. [ABSTRACT FROM AUTHOR]

Aim To assess association between urinary levels of adiponectin and severity of renal involvement in SLE patients. Also, this study aims to determine the value of urinary adiponectin levels to discriminate renal involvement in these patients. Methods In a multi-center cross-sectional survey, 50 consecutive patients diagnosed as having systemic lupus erythematosus ( SLE) according to American College of Rheumatology criteria were classified into two groups with or without renal involvement (microscopic hematuria, reduced glomerular filtration rate < 25% of normal value, and proteinuria > 500 mg/24 h) which was confirmed by renal biopsy. Urinary adiponectin was measured by enzyme-linked immunosorbent assay. SLE disease activity levels were assessed by SLE Disease Activity Index ( SLEDAI) score. Results Comparing urinary levels of adiponectin between the two groups indicated considerable discrepancy in this index between the groups with and without renal involvement (146.33 ± 258.83 ng/ mL vs. 22.96 ± 44.33 ng/ mL, P = 0.023). Also, urinary adiponectin/creatinine ratio was significantly higher in the former group (221.72 ± 414.58 vs. 19.99 ± 41.19, P = 0.019). Our study showed a higher mean SLEDAI score in those with renal involvement than others (23.60 ± 2.53 vs. 9.12 ± 3.03, P < 0.001). Multivariable linear regression analysis with the presence of potential confounders showed that the level of urinary adiponectin was significantly higher in those with renal involvement than other patients (β = 0.470, P = 0.023). The optimal cut-off point for urinary adiponectin levels to discriminate renal involvement from normal renal state was 7.5 ng/mL, yielding a sensitivity of 80% and specificity of 52%. Conclusion Urinary levels of adiponectin are significantly elevated in SLE patients with renal involvement. The measurement of this biomarker can be helpful to discriminate impaired from normal renal function in SLE patients. [ABSTRACT FROM AUTHOR]

Vitamin D3 has a role in many autoimmune diseases and appears to play a function in controlling Rheumatoid Arthritis (RA). The aim of this study is to evaluate the relationship between serum level of vitamin D and RA disease activity score. The serum level of vitamin D in 75 RA patients referred to the rheumatology clinic of Rasoul-Akram hospital was measured. Patients were classified into low, moderate and high RA activity groups based on the DAS-28 criteria (Disease Activity Score in 28 joints) and the mean values of serum vitamin D were compared between the three groups. The mean serum levels of vitamin D in high activity group (17.057±7.7 mg/ml) was significantly less than moderate (30.5±11.3 mg/ml) and low (36.7±19.5 mg/ml) activity groups (P<0.001). The outcome of this study shows that serum level of vitamin D is inversely correlated with the activity of RA. [ABSTRACT FROM AUTHOR]