The importance of tubuloreticular inclusions in lupus nephritis

Nossent, Jiri; Raymond, Warren; Ognjenovic, Milica; Kang, Alexandra; Chakera, Aron; Wong, Daniel

doi:10.1016/j.pathol.2019.07.007

Cited by 5 publications

(6 citation statements)

References 32 publications

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“…The pathological findings we observed correspond to described DM pathological features, as the presence of a perimysial inflammatory infiltrate see in all cases, the C5b-9 deposition on capillaries, even if it is not always specific for DM (31) and the positive DM-specific MxA immunostaining in the majority of cases (72%) and in the same range as reported in DM (19,30). In addition, 64% of patients presented tubuloreticular inclusions, which have been described in DM (25) but also in other immune-mediated diseases (20,26,29). Moreover, we observed a dilatation of blood vessels in the perimysium in the majority of cases, as previously reported (19), as well as the capillaries enlargement which is usual in DM (8,32).…”

Section: Discussionmentioning

confidence: 59%

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

et al. 2020

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Aims: Edema of the limbs is uncommon in idiopathic inflammatory myopathies (IIM). The few reported cases have been associated with severe and refractory dermatomyositis (DM), sometimes in association with cancers. We aimed to determine if edematous myositis is a homogeneous subtype based on clinical, serological and pathological features. Methods: This is a retrospective observational study performed between 2008 and 2015 in the French national referral center for myositis. All adult patients with an inflammatory muscle biopsy and upper limbs edema were included as well as IIM cases without limb edema as controls. Clinical, biological and pathological features were collected. Results: Seventeen edematous myositis were included and compared to 174 IIM without edema, including 50 DM controls. Edema was the first manifestation in 23% of patients. Muscle weakness was severe and symmetric, 71% of patients presented dysphagia and a restrictive ventilatory pattern was found in 40%. Fifty‐two percent of patients had a typical DM skin rash and 23% had cancer within 3 years of diagnosing myositis. Fifty‐three percent of patients presented a myositis specific antibody and only DM‐specific antibodies were detected. Classic pathological DM features (perifascicular atrophy, perifascicular/perimysial perivascular inflammation) were uncommon but capillary C5b‐9 deposition and MxA expression were seen in 79% and 73% of cases, respectively. A perimysial edema was found in 82% of cases. Seventeen percent of patients died (median follow up of 18 months). Edematous myositis demonstrated more marked capillary C5b‐9 deposition compared to IIM controls. There was no clinical, biological or pathological difference with DM controls except for limb edema. Conclusion: Our study underlines that limb edema could be a symptom of IIM and that edematous myositis are mostly DM. The vasculopathy seems to play a key role in its pathophysiology. Limb edema associated with muscle impairment should suggest the diagnosis of DM in clinical settings.

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Section: Discussionmentioning

confidence: 59%

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

et al. 2020

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“…Transmission electron microscopic (TEM) evaluation of biopsy specimens is currently also recommended because it provides further valuable details of intracellular structure, such as the presence of TRIs [ 10 ]. These are subcellular structures located in the cisternae of the endoplasmic reticulum, and their formation was linked to exposure to excess endogenous or exogenous interferon (IFN) [ 11 , 12 ]. The presence of TRIs in LN is not a specific finding as they commonly occur in viral diseases.…”

Section: Discussionmentioning

confidence: 99%

The role of assessment of renal biopsy in electron microscopy in making a diagnosis of juvenile-onset systemic lupus erythematosus in a 16-year-old female patient with depression and proteinuria – case report

Koszutski¹,

Wiernik²,

Hyla-Klekot³

et al. 2022

cejoi

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The course of juvenile-onset systemic lupus erythematosus may vary, from rapid multiorgan involvement to insidious development mimicking different medical conditions. depressive disorder in adolescents poses considerable diagnostic difficulties due to the natural tendency to lowered mood in this age group. However, it may also be the manifestation of a systemic disease. We present a case of a 16-year-old female patient without any somatic symptoms in whom severe depression resistant to treatment was the preceding symptom of juvenile-onset systemic lupus erythematosus (jsLe). Because of isolated proteinuria and presence of antinuclear antibodies, renal biopsy was performed. Light microscopy showed only findings characteristic for membranous nephropathy. examination on electron microscopy showed characteristic tubuloreticular inclusions (Tris) which were crucial for making the diagnosis of systemic lupus erythematosus. The evaluation of renal biopsy specimens by electron microscopy could be a useful diagnostic step to confirm the diagnosis, especially in difficult cases where the criteria for sLe are not fully met. The association of mental symptoms with systemic lupus erythematosus and other autoimmune disorders is well documented. However, the increasing prevalence of depression in children and adolescents poses a risk of delaying the diagnosis of a systemic disease.

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“…Compared to the average cryoglobulinemic GN, kidney biopsies in lupus nephritis contain more electron-dense deposits and much less monocytes/macrophages. Tubuloreticular structures are another typical finding of lupus nephritis [ 41 ]. Whether the “fingerprint-substructures” seen in some deposits by EM hint at the deposition of cryoglobulins is debated.…”

Section: Differential Diagnosismentioning

confidence: 99%

Renal Disease in Cryoglobulinemia

Menter¹,

Hopfer²

2021

Glomerular Dis

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Background: Renal disease in cryoglobulinemia is difficult to grasp and diagnose because it is rare, serological testing is challenging and prone to artifacts, and its morphology is shared by other renal diseases resulting in a spectrum of differential diagnoses. On occasion, a definitive diagnosis cannot even be rendered after immunofluorescence and electron microscopic studies. Summary: Based on kidney biopsies seen in our routine diagnostic and referral practice, we discuss and illustrate various morphological patterns of renal injury associated with cryoglobulins. We outline key pathophysiologic and clinical aspects associated with cryoglobulinemia induced renal disease and describe morphologic changes with a focus on electron microscopy. We present our practical, morphology-based approach to diagnostic decision-making with special consideration of differential diagnoses and disease mimickers. Since cryoglobulins are rarely tested for prior to kidney biopsy, pathologists and clinicians alike must have a high level of suspicion when interpreting renal biopsies and managing patients. Key Messages: Cryoglobulinemia-associated glomerulonephritis (GN) is a multifactorial disease which is important to recognize for clinical practice. Morphological features suggestive of cryoglobulinemia-associated GN include a pattern of membranoproliferative GN with abundance of monocytes and the presence of (pseudo)thrombi. By electron microscopy, the main diagnostic features are a prominent infiltration of monocytes/macrophages and the presence of mesangial and subendothelial deposits with frequently curved microtubular/cylindrical and annular substructures.

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The importance of tubuloreticular inclusions in lupus nephritis

Cited by 5 publications

References 32 publications

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

The role of assessment of renal biopsy in electron microscopy in making a diagnosis of juvenile-onset systemic lupus erythematosus in a 16-year-old female patient with depression and proteinuria – case report

Renal Disease in Cryoglobulinemia

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