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    v1.0.28
    OpenPayerBilling CodesSystemic connective tissue disordersICD-10-CM m31-other-necrotizing-vasculopathies
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    ICD-10-CM Code For Other Necrotizing Vasculopathies: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group M31 includes necrotizing vasculopathies such as necrotizing vasculitis and related disorders that involve inflammation and necrosis of vessel walls, often affecting small and medium vessels and multiple organ systems. These ICD-10-CM diagnosis codes are used to document clinical presentations ranging from isolated cutaneous involvement to systemic disease with organ ischemia, capturing disease severity, organ involvement, or clinical complexity relevant to this group. Accurate coding within the ICD-10-CM M31 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM M31: Other Necrotizing Vasculopathies Overview

    The ICD-10-CM M31 group includes necrotizing vasculopathies characterized by inflammation and necrosis of blood vessel walls, often affecting medium and small vessels and sometimes involving multiple organs. These conditions target the vascular system and can cause ischemia or infarction in skin, nerves, kidneys, and other tissues. Accurate coding matters for reimbursement because it captures disease severity, organ involvement, and the need for specialized therapies, which informs claim adjudication and payment.

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    When to Use

    Use a code from this group when clinical documentation indicates necrotizing inflammation of blood vessels consistent with a necrotizing vasculopathy, specifying the vessel size and organ involvement when available. Select the most specific code that reflects whether the condition is cutaneous, systemic, or associated with other specified underlying disorders.

    ICD-10-CM Codes

    13 rows

    ICD-10-CM CodeDescription
    M31.0Hypersensitivity angiitis
    M31.10Thrombotic microangiopathy, unspecified
    M31.11Hematopoietic stem cell transplantation-associated thrombotic microangiopathy [HSCT-TMA]
    M31.19Other thrombotic microangiopathy
    M31.2Lethal midline granuloma
    M31.30Wegener's granulomatosis without renal involvement
    M31.31Wegener's granulomatosis with renal involvement
    M31.4Aortic arch syndrome [Takayasu]
    M31.5Giant cell arteritis with polymyalgia rheumatica
    M31.6Other giant cell arteritis
    M31.7Microscopic polyangiitis
    M31.8Other specified necrotizing vasculopathies
    M31.9Necrotizing vasculopathy, unspecified

    DRG Codes

    9 rows

    DRG CodeDescription
    299Peripheral Vascular Disorders with MCC
    300Peripheral Vascular Disorders with CC
    301Peripheral Vascular Disorders without CC/MCC
    542Pathological Fractures and Musculoskeletal and Connective Tissue Malignancy with MCC
    543Pathological Fractures and Musculoskeletal and Connective Tissue Malignancy with CC
    544Pathological Fractures and Musculoskeletal and Connective Tissue Malignancy without CC/MCC
    545Connective Tissue Disorders with MCC
    546Connective Tissue Disorders with CC
    547Connective Tissue Disorders without CC/MCC

    Typical Clinical Scenarios

    • An older adult presents to the emergency department with sudden-onset digital ischemia, painful fingertip ulcers, and livedo racemosa after a recent respiratory infection; vascular imaging shows segmental medium-vessel occlusions with histopathology demonstrating transmural necrotizing inflammation. The acute presentation with systemic signs and biopsy-proven necrotizing vasculitis leads to assignment of a code for acute necrotizing arteritis involving medium-sized vessels. Typical codes: M31.0, M31.2
    • A middle-aged patient in rheumatology clinic with known autoimmune disease has progressive renal impairment, new-onset mononeuritis multiplex, and constitutional symptoms over months; nerve and kidney biopsies reveal chronic necrotizing vasculopathy with granulomatous features. This chronic, systemic course with biopsy confirmation and multisystem involvement prompts use of codes for chronic or unspecified necrotizing vasculopathies. Typical codes: M31.7, M31.8
    • A hospitalized patient develops skin necrosis and digital gangrene as a complication during treatment for an underlying connective tissue disorder, and clinicians document thrombotic microangiopathy with necrotizing changes attributed to vasculopathy rather than infection. In this complication/comorbidity scenario the clinical team assigns a code reflecting necrotizing vasculopathy presenting as a complication of systemic disease or as an acute necrotizing vascular lesion. Typical codes: M31.1, M31.31

    Related Codes

    M30-POLYARTERITIS-NODOSA-AND-RELATED-CONDITIONS

    ICD-10-CM Code For Polyarteritis Nodosa And Related Conditions: Group Overview

    M32-SYSTEMIC-LUPUS-ERYTHEMATOSUS

    ICD-10-CM Code For Systemic Lupus Erythematosus SLE: Group Overview

    M33-DERMATOPOLYMYOSITIS-CONDITIONS

    ICD-10-CM Code For Dermatopolymyositis: Group Overview

    M34-SYSTEMIC-SCLEROSIS-SCLERODERMA

    ICD-10-CM Code For Systemic Sclerosis [Scleroderma]: Group Overview

    M35-OTHER-SYSTEMIC-INVOLVEMENT-OF-CONNECTIVE-TISSUE

    ICD-10-CM Code For Other Systemic Involvement Of Connective Tissue: Group Overview

    M36-SYSTEMIC-CONNECTIVE-TISSUE-DISORDERS

    ICD-10-CM Code For Systemic Disorders Of Connective Tissue In Diseases Classified Elsewhere: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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