A Case of Acute Renal Failure Associated with IgG4-Related Disease Presenting Both Tubulointerstitial Nephritis and Retroperitoneal Fibrosis

We report a case of IgG4-related disease presenting both tubulointerstitial nephritis and retroperitoneal fibrosis causing acute renal failure in a 63-year-old male. He was admitted to our hospital because of acute renal failure requiring emergent hemodialysis. Computed tomography showed a soft-tissue density mass with an irregular border in the retroperitoneum. The mass involved bilateral ureters and had caused acute renal failure by bilateral hydronephrosis. Because of a history of uveitis and high IgG4 levels, we considered a diagnosis of retroperitoneal fibrosis, IgG4-related disease. Kidney biopsy revealed IgG4-related kidney disease with interstitial nephritis. After relief of urinary obstruction by inserting ureteral catheters into the bilateral ureters, renal function recovered.

Tubulointerstitial Nephritis Complicated with Primary Sjogren’s Syndrome under Treatment for Type 2 Diabetes Mellitus

Tubulointerstitial nephritis complicated by primary Sj?gren’s syndrome in a patient under treatment for type 2 diabetes mellitus was diagnosed in the early stage of the disease by renal biopsy. The symptoms of primary Sj?gren’s syndrome, such as thirst and polydipsia, were masked by the characteristic symptoms of type 2 diabetes mellitus. An association between sicca symptoms and diabetes mellitus (types 1 and 2) has been previously reported. Hence, it is possible that there are common underlying immunological mechanisms between primary Sj?gren’s syndrome and diabetes mellitus of both types. Intervention with steroids in a timely manner appears to have prevented or slowed the progression of re- nal impairment.

Renal Involvement in Sarcoidosis

Introduction: Sarcoidosis is a granulomatous systemic disease. Renal damage is rare but it can evolve to chronic renal failure (CRF). The aim of our work is to describe the clinical, paraclinical, histological and evolutionary profile of renal involvement during sarcoidosis and to identify the progression factors leading to CRF. Materials and Methods: This is a retrospective descriptive study from January 2009 to December 2022. We collected the medical records of patients with sarcoidosis and renal involvement. To study the progression factors leading to CRF we identified two groups of patients: the group with normal renal function and the group that developed CRF. Results: We included in our study 17 patients with renal sarcoidosis. Their mean age was 45 ± 14.7 years and the sex ratio was 0.4. Renal involvement is revealing of sarcoidosis in 76% of cases. Renal failure was diagnosed in 88% of patients. Proteinuria was positive in 76.5% of cases, leukocyturia in 59% of patients and hematuria in 41% of patients. Renal biopsy was performed in 15 patients showing tubulointerstitial nephropathy in all biopsies. Epithelioid and gigan-to-cellular granuloma without caseous necrosis was found in 46% of cases. Associated glomerular involvement such as segmental and focal hyalinosis was found in 2 patients. Corticosteroid therapy was initiated in 88% of patients for a median duration of 15 months. Normalization of renal function was achieved in 41% of patients, while 59%, i.e. 10 patients, retained a CRF, including 2 who were on dialysis. We showed a statistically significant relationship between the evolution towards CRF and the presence of interstitial fibrosis > 25%. Conclusion: Despite its rarity, renal involvement can be revealing of sarcoidosis, which can condition the prognosis and lead to CRF. Its detection allows an early diagnosis and treatment.

End Stage Renal Disease in a Child with Epidermolysis Bullosa

Epidermolysis bullosa (EB) is an inherited connective tissue disease causing blisters in the skin and mucosal membranes. In severe cases, EB may be associated with renal damage through several mechanisms, mainly immunological ones. The present case described a young male with dystrophic recessive EB who developed an advanced chronic renal damage secondary to tubulointerstitial nephritis that was demonstrated by a renal biopsy. Unpublished previously, this complication should be considered among the possible causes of renal damage in EB. Also it is recommended a protocoled surveillance of renal and urinary tract complications in children with EB.

Systemic Capillary Leak Syndrome Secondary to COVID-19 Infection: Case Report

Introduction: Systemic capillary leak syndrome (SCLS) is an increasingly recognized rare syndrome. Its diagnosis is suggested by the occurrence of edema with arterial hypotension, hemoconcentration, and paradoxical hypoalbuminemia. SCLS can be idiopathic (Clarkson syndrome) or secondary. Secondary SCLS (SSCLS) is mainly triggered by infections (especially viruses), drugs (antitumor therapy), malignancies, and inflammatory diseases. We report a case of systemic capillary leak syndrome secondary to the COVID-19 infection. Observation: A 74-year-old chronic smoker with no particular history was initially admitted to the intensive care unit (ICU) with a picture of respiratory distress secondary to a COVID-19 infection with favorable evolution, hence his transfer to the emergency services. On Day 8 of hospitalization, following the installation of arterial hypotension, not responding to filling, associated with hypoalbuminemia, and generalized edematous syndrome, and in the absence of any other explanation for this clinical picture, a SCLS secondary to COVID-19 infection was suggested. On the balance sheet, after the discovery of acute renal failure, serum creatinine went from 7.9 mg/l to 16.6 mg/l with microalbuminuria at 420 mg/24h and leukocyturia at 20 elements/mm3 without germ-evoked tubulointerstitial nephritis (TIN) secondary to a viral infection with COVID-19. The evolution was marked by the spontaneous regression of the edema and the normalization of the blood pressure figures. Discussion: The classic triad combining hypotension, hemoconcentration, and hypoalbuminemia suggests the diagnosis of SCLS once all other causes of shock have been ruled out. Hemoconcentration is less constant in SSCLS than in ISCLS. This is the case with our patient. The exact pathophysiological process of SCLS is largely unknown. Viral infections are the most common infectious cause of SCLS. The kidneys are the second-most common organs affected by the SARS-Cov-2 coronavirus infection. The presence of nephritis can be used as an indicator of SCLS, which can be a predictor of serious complications such as fluid overload, respiratory failure, and the need for ICU admission. Conclusion: In the event of COVID-19 infection, the appearance of hypotension and hypoalbuminemia with the gradual onset of generalized edema should suggest SCLS. The establishment of close monitoring is mandatory, given the risk of fatal evolution. Fortunately, for our patient, the evolution was favorable.