Systemic connective tissue disorders (M30-M36)
M30Polyarteritis nodosa and related conditions
Excludes 1: microscopic polyarteritis (M31.7)
M30.0Polyarteritis nodosa
M30.1Polyarteritis with lung involvement [Churg-Strauss]
› Allergic granulomatous angiitis
› Eosinophilic granulomatosis with polyangiitis [EGPA]
M30.2Juvenile polyarteritis
M30.3Mucocutaneous lymph node syndrome [Kawasaki]
M30.8Other conditions related to polyarteritis nodosa
› Polyangiitis overlap syndrome
M31Other necrotizing vasculopathies
M31.0Hypersensitivity angiitis
› Goodpasture's syndrome
M31.1Thrombotic microangiopathy
M31.10Thrombotic microangiopathy, unspecified
M31.11Hematopoietic stem cell transplantation-associated thrombotic microangiopathy [HSCT-TMA]
› Transplant-associated thrombotic microangiopathy [TA-TMA]
Code First: if applicable:
› complications of bone marrow transplant (T86.0-)
› complications of stem cell transplant (T86.5)
Use Additional Code: code to identify specific organ dysfunction, such as:
› acute kidney failure (N17.-)
› acute respiratory distress syndrome (J80)
› capillary leak syndrome (I78.8)
› diffuse alveolar hemorrhage (R04.89)
› encephalopathy (metabolic) (septic) (G93.41)
› fluid overload, unspecified (E87.70)
› graft versus host disease (D89.81-)
› hemolytic uremic syndrome (D59.3-)
› hepatic failure (K72.-)
› hepatic veno-occlusive disease (K76.5)
› idiopathic interstitial pneumonia (J84.11-)
› sinusoidal obstruction syndrome (K76.5)
M31.19Other thrombotic microangiopathy
› Thrombotic thrombocytopenic purpura
M31.2Lethal midline granuloma
M31.3Wegener's granulomatosis
› Granulomatosis with polyangiitis
› Necrotizing respiratory granulomatosis
M31.30Wegener's granulomatosis without renal involvement
› Wegener's granulomatosis NOS
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
› Microscopic polyarteritis
Excludes 1: polyarteritis nodosa (M30.0)
M31.8Other specified necrotizing vasculopathies
› Hypocomplementemic vasculitis
› Septic vasculitis
M31.9Necrotizing vasculopathy, unspecified
M32Systemic lupus erythematosus (SLE)
Excludes 1: lupus erythematosus (discoid) (NOS) (L93.0)
M32.0Drug-induced systemic lupus erythematosus
Use Additional Code: code for adverse effect, if applicable, to identify drug (T36-T50) with fifth or sixth character 5)
M32.1Systemic lupus erythematosus with organ or system involvement
M32.10Systemic lupus erythematosus, organ or system involvement unspecified
M32.11Endocarditis in systemic lupus erythematosus
› Libman-Sacks disease
M32.12Pericarditis in systemic lupus erythematosus
› Lupus pericarditis
M32.13Lung involvement in systemic lupus erythematosus
› Pleural effusion due to systemic lupus erythematosus
M32.14Glomerular disease in systemic lupus erythematosus
› Lupus renal disease NOS
M32.15Tubulo-interstitial nephropathy in systemic lupus erythematosus
M32.19Other organ or system involvement in systemic lupus erythematosus
Use Additional Code: code(s) to identify organ or system involvement, such as encephalitis (G05.3)
M32.8Other forms of systemic lupus erythematosus
M32.9Systemic lupus erythematosus, unspecified
› SLE NOS
› Systemic lupus erythematosus NOS
› Systemic lupus erythematosus without organ involvement
M33Dermatopolymyositis
M33.0Juvenile dermatomyositis
M33.00Juvenile dermatomyositis, organ involvement unspecified
M33.01Juvenile dermatomyositis with respiratory involvement
M33.02Juvenile dermatomyositis with myopathy
M33.03Juvenile dermatomyositis without myopathy
M33.09Juvenile dermatomyositis with other organ involvement
M33.1Other dermatomyositis
› Adult dermatomyositis
M33.10Other dermatomyositis, organ involvement unspecified
M33.11Other dermatomyositis with respiratory involvement
M33.12Other dermatomyositis with myopathy
M33.13Other dermatomyositis without myopathy
› Dermatomyositis NOS
M33.19Other dermatomyositis with other organ involvement
M33.2Polymyositis
M33.20Polymyositis, organ involvement unspecified
M33.21Polymyositis with respiratory involvement
M33.22Polymyositis with myopathy
M33.29Polymyositis with other organ involvement
M33.9Dermatopolymyositis, unspecified
M33.90Dermatopolymyositis, unspecified, organ involvement unspecified
M33.91Dermatopolymyositis, unspecified with respiratory involvement
M33.92Dermatopolymyositis, unspecified with myopathy
M33.93Dermatopolymyositis, unspecified without myopathy
M33.99Dermatopolymyositis, unspecified with other organ involvement
M34Systemic sclerosis [scleroderma]
Excludes 1: circumscribed scleroderma (L94.0)
› neonatal scleroderma (P83.88)
M34.0Progressive systemic sclerosis
M34.1CR(E)ST syndrome
› Combination of calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia
M34.2Systemic sclerosis induced by drug and chemical
Code First: poisoning due to drug or toxin, if applicable (T36-T65 with fifth or sixth character 1-4)
Use Additional Code: code for adverse effect, if applicable, to identify drug (T36-T50) with fifth or sixth character 5)
M34.8Other forms of systemic sclerosis
M34.81Systemic sclerosis with lung involvement
Code Also: if applicable:
› other interstitial pulmonary diseases (J84.89)
› secondary pulmonary arterial hypertension (I27.21)
M34.82Systemic sclerosis with myopathy
M34.83Systemic sclerosis with polyneuropathy
M34.89Other systemic sclerosis
M34.9Systemic sclerosis, unspecified
M35Other systemic involvement of connective tissue
Excludes 1: reactive perforating collagenosis (L87.1)
M35.0Sjögren syndrome
› Sicca syndrome
Use Additional Code: code to identify associated manifestations
Excludes 1: dry mouth, unspecified (R68.2)
M35.00Sjögren syndrome, unspecified
M35.01Sjögren syndrome with keratoconjunctivitis
M35.02Sjögren syndrome with lung involvement
M35.03Sjögren syndrome with myopathy
M35.04Sjögren syndrome with tubulo-interstitial nephropathy
› Renal tubular acidosis in sicca syndrome
M35.05Sjögren syndrome with inflammatory arthritis
M35.06Sjögren syndrome with peripheral nervous system involvement
M35.07Sjögren syndrome with central nervous system involvement
M35.08Sjögren syndrome with gastrointestinal involvement
M35.0ASjögren syndrome with glomerular disease
M35.0BSjögren syndrome with vasculitis
M35.0CSjögren syndrome with dental involvement
M35.09Sjögren syndrome with other organ involvement
M35.1Other overlap syndromes
› Mixed connective tissue disease
Excludes 1: polyangiitis overlap syndrome (M30.8)
M35.2Behçet's disease
M35.3Polymyalgia rheumatica
Excludes 1: polymyalgia rheumatica with giant cell arteritis (M31.5)
M35.4Diffuse (eosinophilic) fasciitis
M35.5Multifocal fibrosclerosis
M35.6Relapsing panniculitis [Weber-Christian]
Excludes 1: lupus panniculitis (L93.2)
› panniculitis NOS (M79.3-)
M35.7Hypermobility syndrome
› Familial ligamentous laxity
Excludes 1: ligamentous laxity, NOS (M24.2-)
Excludes 2: Ehlers-Danlos syndromes (Q79.6-)
M35.8Other specified systemic involvement of connective tissue
M35.81Multisystem inflammatory syndrome
› MIS-A
› MIS-C
› Multisystem inflammatory syndrome in adults
› Multisystem inflammatory syndrome in children
› Pediatric inflammatory multisystem syndrome
› PIMS
Code First: , if applicable, COVID-19 (U07.1)
Code Also: any associated complications such as:
› acute hepatic failure (K72.0-)
› acute kidney failure (N17.-)
› acute myocarditis (I40.-)
› acute respiratory distress syndrome (J80)
› cardiac arrhythmia (I47-I49.-)
› pneumonia due to COVID-19 (J12.82)
› severe sepsis (R65.2-)
› viral cardiomyopathy (B33.24)
› viral pericarditis (B33.23)
Use Additional Code: code, if applicable, for:
› exposure to COVID-19 or SARS-CoV-2 infection (Z20.822)
› personal history of COVID-19 (Z86.16)
› post COVID-19 condition (U09.9)
M35.89Other specified systemic involvement of connective tissue
M35.9Systemic involvement of connective tissue, unspecified
› Autoimmune disease (systemic) NOS
› Collagen (vascular) disease NOS
M36Systemic disorders of connective tissue in diseases classified elsewhere
Excludes 2: arthropathies in diseases classified elsewhere (M14.-)
M36.0Dermato(poly)myositis in neoplastic disease
Code First: underlying neoplasm (C00-D49)
M36.1Arthropathy in neoplastic disease
Code First: underlying neoplasm, such as:
› leukemia (C91-C95)
› malignant histiocytosis (C96.A)
› multiple myeloma (C90.0)
M36.2Hemophilic arthropathy
› Hemarthrosis in hemophilic arthropathy
Code First: underlying disease, such as:
› factor VIII deficiency (D66)
› with vascular defect (D68.0-)
› factor IX deficiency (D67)
› hemophilia (classical) (D66)
› hemophilia B (D67)
› hemophilia C (D68.1)
M36.3Arthropathy in other blood disorders
M36.4Arthropathy in hypersensitivity reactions classified elsewhere
Code First: underlying disease, such as:
› Henoch (-Schönlein) purpura (D69.0)
› serum sickness (T80.6-)
M36.8Systemic disorders of connective tissue in other diseases classified elsewhere
Code First: underlying disease, such as:
› alkaptonuria (E70.29)
› hypogammaglobulinemia (D80.-)
› ochronosis (E70.29)