An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries

Patil, Anil Kumar; Prabhakar, Appaswamy Thirumal; Sivadasan, Ajith; Alexander, Mathew; Chacko, Geeta

doi:10.4103/0028-3886.152642

Cited by 3 publications

(3 citation statements)

References 5 publications

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“…Those patients who do not respond to the first-line therapy should receive IVIG in association with corticosteroids. Similar results, showing an improvement of both muscle and skin disease, have been reported in various forms of inflammatory myopathies as polymyositis , juvenile dermatomyositis [ 40 ], or necrotizing myopathy [ 41 ]. Moreover IVIG treatment affects well the dermatomyositis complications as ulcerative skin lesions and calcinosis cutis [ 42 ].…”

Section: Connective Tissue Diseasessupporting

confidence: 84%

Intravenous Immunoglobulins: Mode of Action and Indications in Autoimmune and Inflammatory Dermatoses

Dourmishev

Guleva

Miteva

2016

International Journal of Inflammation

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Intravenous immunoglobulins (IVIGs), a mixture of variable amounts of proteins (albumin, IgG, IgM, IgA, and IgE antibodies), as well as salt, sugar, solvents, and detergents, are successfully used to treat a variety of dermatological disorders. For decades, IVIGs have been administered for treatment of infectious diseases and immune deficiencies, since they contain natural antibodies that represent a first-line defense against pathogens. Today their indication has expanded, including the off-label therapy for a variety of autoimmune and inflammatory diseases. In dermatology, IVIGs are administered for treatment of different disorders at different therapeutic regimens, mostly with higher doses then those administered for treatment of infectious diseases. The aim of this prospective review is to highlight the indications, effectiveness, side effects, and perspectives of the systemic treatment with IVIGs for patients with severe, life-threatening, and resistant to conventional therapies autoimmune or inflammatory dermatoses.

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Section: Connective Tissue Diseasessupporting

confidence: 84%

Intravenous Immunoglobulins: Mode of Action and Indications in Autoimmune and Inflammatory Dermatoses

Dourmishev

Guleva

Miteva

2016

International Journal of Inflammation

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“…11,14 Skin manifestations occur in less than 10% of patients with IMNM, 2 including heliotrope rash, Gottron papules, V-sign, Shawl sign, Mechanic's hands, and skin hyperpigmentation. [15][16][17] Erythema nodosum (EN) is a common form of acute nodular septal panniculitis without signs of vasculitis. [18][19][20][21][22] It is clinically characterized by bilaterally symmetrical red and painful subcutaneous nodules, mainly localized to the lower extremities, pretibial areas especially.…”

Section: Introductionmentioning

confidence: 99%

“…Skin manifestations occur in less than 10% of patients with IMNM, 2 including heliotrope rash, Gottron papules, V-sign, Shawl sign, Mechanic’s hands, and skin hyperpigmentation. 15 – 17 …”

Section: Introductionmentioning

confidence: 99%

<p>Immune-Mediated Necrotizing Myopathy Initially Presenting as Erythema Nodosum</p>

Ying¹,

Li²,

Tang³

et al. 2020

JIR

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Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by severe diffuse proximal myofiber necrosis in the context of inflammatory myopathy. Autoantibodies of anti-signal recognition particle and anti-hydroxy-3-methylglutaryl-CoA reductase are two antibodies specific to IMNM. Erythema nodosum (EN) is often accompanied by various systemic diseases, such as autoimmune diseases. Herein, we report a female patient with signal recognition particle-associated IMNM, with EN as the first presentation. She showed significant clinical improvement after the initiation of glucocorticoids, intravenous immunoglobulin, rituximab, and mycophenolate mofetil. This case indicates that IMNM can initially present as EN. IMNM and EN might have overlapping pathogeneses.

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Rare case of Sjögren’s syndrome antibody (SSA-Ro52kDA)-associated interstitial lung disease and myositis with pipe stem capillaries

2021

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Necrotising myopathy with pipestem capillaries is a distinct form of inflammatory myopathy exhibiting only sparse inflammation on biopsy, with clinical presentation and histopathological profile entirely different from dermatomyositis, polymyositis or inclusion body myositis. A 51-year-old non-diabetic man presents with progressively worsening shortness of breath and myalgias with only mild proximal muscle weakness and elevated serum creatine kinase. Autoimmune workup, ordered after ruling out infectious and cardiac aetiologies, returned positive for Sjögren’s syndrome antibody (SSA/Ro-52). Lung imaging and biopsy were suggestive of cryptogenic organising pneumonia and muscle biopsy showed myositis with pipestem capillaries and abnormal deposition of membrane attack complex with only sparse inflammation. The patient received high-dose steroids, mycophenolate mofetil, intravenous immunoglobulin and rituximab with improvement in muscle symptoms. However, his pulmonary findings progressed, requiring evaluation for a lung transplant. This case emphasises the need for further research to better understand this disease entity and improve mortality and morbidity in these patients.

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An unusual case of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries

Cited by 3 publications

References 5 publications

Intravenous Immunoglobulins: Mode of Action and Indications in Autoimmune and Inflammatory Dermatoses

Intravenous Immunoglobulins: Mode of Action and Indications in Autoimmune and Inflammatory Dermatoses

<p>Immune-Mediated Necrotizing Myopathy Initially Presenting as Erythema Nodosum</p>

Rare case of Sjögren’s syndrome antibody (SSA-Ro52kDA)-associated interstitial lung disease and myositis with pipe stem capillaries

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