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primary hiv infection can be identified by recent appearance of hiv antibody or by identifying viral products (hiv-rna or hiv-dna and/or ultrasensitive hiv p24 antigen) with negative (or weakly reactive) hiv antibody.
human immunodeficiency virus disease associated with generalised lymphadenopathy this is a lentivirus (slowly replicating retrovirus) that causes acquired immunodeficiency syndrome (aids), a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cance...
acute human immunodeficiency virus infection syndrome this syndrome is the initial manifestation of human immunodeficiency virus (hiv) infection. one half to two thirds of patients present with an acute "mononucleosis-type" illness (fever, swollen lymph nodes, sore throat, rash, headache, malaise, arthralgia, or myalg...
symptomatic hiv infection is mainly caused by the emergence of certain opportunistic infections that the immune system would normally prevent. this stage of hiv infection is often characterised by multi-system disease and infections can occur in almost all body systems.
hiv-associated immune reconstitution inflammatory syndrome immune reconstitution inflammatory syndrome (iris) results from pathological immune responses occurring during immune reconstitution and is strongly associated with antiretroviral therapy for hiv infection and aids. there is a wide range of clinical manifestat...
human immunodeficiency virus disease associated with haematological or immunological abnormalities this is a lentivirus (slowly replicating retrovirus) that causes acquired immunodeficiency syndrome (aids), a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic in...
human immunodeficiency virus disease associated with lymphoid interstitial pneumonitis this is a lentivirus (slowly replicating retrovirus) that causes acquired immunodeficiency syndrome (aids), a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and...
human immunodeficiency virus disease enteritis this is an inflammation of small intestine associated with infection with human immunodeficiency virus (hiv). in diarrhoea, a major complication of human immunodeficiency virus (hiv) disease, the etiology of which is often obscure.
gastritis due to human immunodeficiency virus disease this condition is the inflammation of the stomach due to hiv disease.
myelitis due to human immunodeficiency virus is described as an inflammatory disorder of the spinal cord due to infection by the hiv virus. the symptoms and signs may be acute or subacute in onset, and include paraparesis or paraplegia, sensory loss appropriate with the level of the spinal cord affected, and loss of sp...
hiv - [human immunodeficiency virus] disease associated with burkitt lymphoma burkitt lymphoma attributable to co-infection with epstein-barr virus and human immunodeficiency virus
human immunodeficiency virus disease associated with other types of non-hodgkin lymphoma this is a lentivirus (slowly replicating retrovirus) that causes acquired immunodeficiency syndrome (aids), a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections a...
human immunodeficiency virus disease associated with other malignant neoplasms of lymphoid, haematopoietic or related tissue this is a lentivirus (slowly replicating retrovirus) that causes acquired immunodeficiency syndrome (aids), a condition in humans in which progressive failure of the immune system allows life-th...
human immunodeficiency virus disease associated with multiple malignant neoplasms this is a lentivirus (slowly replicating retrovirus) that causes acquired immunodeficiency syndrome (aids), a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and canc...
human immunodeficiency virus disease associated with encephalopathy this is a lentivirus (slowly replicating retrovirus) that causes acquired immunodeficiency syndrome (aids), a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thrive....
dementia due to human immunodeficiency virus develops during the course of confirmed hiv disease, in the absence of a concurrent illness or condition other than hiv infection that could explain the clinical features. although a variety of patterns of cognitive deficit are possible depending on where the hiv pathogenic...
encephalitis due to human immunodeficiency virus type 1 is described as an inflammatory process of the brain, frequently with evidence of meningeal involvement, due to infection by a human immunodeficiency type 1 virus. the clinical manifestations are usually subacute, with fever and variable combinations of convulsion...
encephalitis due to human immunodeficiency virus type 2 is described as an inflammatory process of the brain, frequently with evidence of meningeal involvement, due to infection by a human immunodeficiency type 2 virus. the clinical manifestations are usually subacute, with fever and variable combinations of convulsion...
human immunodeficiency virus disease associated with wasting syndrome this is a lentivirus (slowly replicating retrovirus) that causes acquired immunodeficiency syndrome (aids), a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thriv...
oesophagitis associated with human immunodeficiency virus disease this is an inflammation of the oesophagus associated with hiv disease. it may include several etiologies (e.g. infection, neoplasm, toxic).
hiv or aids vacuolar myelopathy is described as an inflammatory and degenerative disorder of the spinal cord due to infection by the hiv virus. the posterior and lateral columns are predominantly affected and show vacuolar degeneration. the symptoms and signs are usually subacute of chronic in onset, and include spasti...
congenital human immunodeficiency virus infection is described as a disease affecting neonates, caused by an infection with human immunodeficiency virus in utero. transmission is by vertical transmission. confirmation is by identification of human immunodeficiency virus in the neonate.
secondary agranulocytosis this is a secondary and acute condition involving a severe and dangerous leukopenia (lowered white blood cell count), most commonly of neutrophils causing a neutropaenia in the circulating blood.
drug-induced secondary agranulocytosis this refers to a drug-induced secondary, acute condition involving a severe and dangerous leukopenia (lowered white blood cell count), most commonly of neutrophils causing a neutropaenia in the circulating blood.
toxic secondary agranulocytosis this refers to a toxic, secondary, acute condition involving a severe and dangerous leukopenia (lowered white blood cell count), most commonly of neutrophils causing a neutropaenia in the circulating blood.
neutropaenia associated with t-cell large granular lymphocyte leukaemia t-cell large granular lymphocyte leukemia (t-cell lgl leukemia) is a lymphoproliferative malignancy that arises from the mature t-cell (cd3+) lineage. it is characterised by neutropaenia, anaemia or thrombocytopenia, and moderate lymphocytosis.
adult-onset immunodeficiency is described as adults with disseminated mycobacterial infections and/or other aids-defining infections, often involving concomitant neutrophilic dermatoses. all patients have high titres of anti-interferon-gamma and normal cd4 t helper cell counts.
acquired immunodeficiency due to loss of immunoglobulin s (protein loss) may occur via the gi tract (protein losing enteropathy), via the kidney (nephrotic syndrome) or via the skin (in severe skin damage).
lupus erythematosus is described as an autoimmune non-organ specific inflammatory disease characterised by the presence of antibodies to dna, rna and other components of the nucleus. it has a very variable clinical presentation and course ranging from an acute fulminant life-threatening disorder with involvement of hea...
systemic lupus erythematosus (sle) is a clinically multisystem disease, which is autoimmune in origin and is characterised by the presence of autoantibodies directed against nuclear antigens. manifestations include rash, arthritis and fatigue, nephritis, neurological problems, anaemia and thrombocytopenia at the more ...
lupus glomerulonephritis in systemic lupus erythematosus, immunoglobulin deposits including igg, iga and igm (“full house” with complement components c3 and c1q) are found in sub-endothelial, intra- membranous and sub-epithelial positions. light microscopic appearances in lupus glomerulonephritis are pleomorphic, and ...
systemic lupus erythematosus with skin involvement systemic lupus erythematosus (sle) involving the skin. this may present with a malar "butterfly" erythema or with extensive necrolysis of sun-exposed skin, particularly on the head, neck and upper torso.
immunobullous systemic lupus erythematosus is an often transient autoimmune skin disease characterised by blisters on the skin and, less commonly, mucous membranes occurring in the setting of systemic lupus erythematosus. the exact causes of the disease are unknown but it is mediated by autoantibodies to the basement ...
acute cutaneous lupus erythematosus is closely associated with systemic lupus erythematosus and double-stranded dna antibodies. its hallmark is the “butterfly” rash across the face occurring in half of patients with systemic lupus. there may however be widespread involvement of the skin with patchy erythema, oedema an...
subacute cutaneous lupus erythematosus is a non-scarring form of lupus erythematosus typified by the presence of circulating anti-ro/ssa antibodies and discoid or annular inflamed red patches with variable fine scaling on sun-exposed skin, especially the sides of the face and neck, the vee of the neck, the extensor su...
drug-induced lupus erythematosus is a syndrome in which positive antinuclear antibodies are associated with symptoms, such as fever, malaise, arthritis, intense arthralgia/myalgia, serositis, and/or rash. the syndrome appears during therapy with certain medications (e.g., procainamide, hydralazine, phenytoin) and tumo...
chronic cutaneous lupus erythematosus (le) is characterised by the presence of circumscribed cutaneous plaques showing varying degrees of oedema, erythema, scaling, follicular plugging and atrophy. it most commonly presents as discoid plaques involving the face, ears and scalp but can be widely disseminated or may aff...
discoid lupus erythematosus is characterised by the presence of discoid plaques showing varying degrees of oedema, erythema, scaling, follicular plugging and atrophy. it typically involves the face, ears and scalp, but widespread dissemination may occur. it can cause marked disfigurement with prominent facial scarring...
verrucous lupus erythematosus is described as a disfiguring and often treatment-resistant from of chronic cutaneous lupus erythematosus characterised by marked warty hyperkeratosis of involved skin. if hyperkeratosis is marked, a warty lesion with a red, slightly raised edge results. verrucous lupus erythematosus is mo...
lupus erythematosus tumidus is a dermal form of chronic cutaneous lupus erythematosus. it is characteristically photosensitive and located in sun-exposed sites. it presents with infiltrated erythematous nodules and plaques which become annular. it does not scar and is clinically very similar to jessner lymphocytic fro...
chilblain lupus erythematosus chilblain lupus is characterised by the development of red or dusky purple plaques with scarring and atrophy on the fingers and toes especially; the remainder of the skin of the hands and feet, the nose, ears, elbows and knees may, however, also be affected.
chronic cutaneous lupus erythematosus of scalp this form of lupus erythematosus presents with patchy scarring alopecia, often associated with prominent perifollicular inflammation and scaling around the active edges of the plaques. it may occur in isolation or be associated with discoid lupus elsewhere, especially on ...
lupus panniculitis is characterised by a destructive inflammation of subcutaneous fat. this manifests itself clinically as indurated plaques which resolve with localised fat atrophy. depending on the intensity of the inflammation a patient may first present with atrophy rather than induration. the overlying skin may s...
neonatal lupus erythematosus results from trans-placental transfer of maternal antibodies, in particular anti-ro/ssa and anti la/ssb. it manifests with an erythematous rash which may be obviously photosensitive and is closely associated with congenital heart block. the mother may have known lupus, especially subacute ...
lupus erythematosus of oral mucosa lupus erythematosus affecting oral mucosa. this may be associated with either cutaneous or systemic lupus erythematosus and presents most commonly as mucosal ulceration. histologically there is a lichenoid mucositis.
idiopathic inflammatory myopathy involves these comprise a diverse group of syndromes that have in common persistent muscle inflammation of unknown pathophysiology, resulting in damage that affects muscle function. the inflammatory muscle disease can either be acute or chronic in nature.
dermatomyositis is an inflammatory myopathy, showing progressive, symmetrical muscle weakness, low muscle endurance, and cutaneous manifestations such as gottron’s papules, heliotrope rash, shawl sign, v-sign and mechanic’s hand. internal organ manifestations such as interstitial pneumonia (pneumonitis) and myocarditi...
adult dermatomyositis is a systemic inflammatory disorder affecting the skeletal muscles, the skin, and other organs. it is characterised by symmetric proximal muscle weakness, increased serum muscle enzymes, myopathic changes upon electromyography, typical histological findings on muscle biopsy, and typical dermatolo...
amyopathic dermatomyositis is a variant of dermatomyositis that is characterised by the typical skin rash but without the muscle abnormalities. the amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that ar...
hypomyopathic dermatomyositis is a rare subset of dermatomyositis characterised by a lack of subjective symptoms but with subclinical evidence of muscle inflammation on muscle biopsy, electromyography, or other procedures.
adult dermatomyositis with calcinosis dermatomyositis with calcinosis is an unusual manifestation in adult patients with dermatomyositis. calcinosis cutis is manifested by firm, coloured nodules, commonly over bony prominences, that can extrude through the surface of the skin, leading to infections. calcification of t...
paraneoplastic dermatomyositis dermatomyositis (dm) associated with an underlying solid malignancy has been reported in up to 40% of patients in reported case series. malignancy is much commoner in patients with dm presenting after the age of 40. the range of associated cancers is wide and largely reflects the prevale...
anti-synthetase syndrome with dermatomyositis dermatomyositis associated with autoantibodies to aminoacyl-transfer rna synthetases, which confer a high risk of interstitial lung disease (anti-synthetase syndrome) as well as myositis.
juvenile dermatomyositis is the early-onset form of dermatomyositis, a systemic autoimmune inflammatory muscle disorder, characterised by proximal muscle weakness, evocative skin lesion, and systemic manifestations.
hypomyopathic juvenile dermatomyositis is young-onset subset of dermatomyositis characterised by a lack of subjective symptoms but with subclinical evidence of muscle inflammation on muscle biopsy, electromyography, or other procedures.
juvenile dermatomyositis with cutaneous vasculitis is an early-onset form of dermatomyositis, characterised by proximal muscle weakness, evocative skin lesion, and systemic manifestations, along with cutaneous vasculitis and ulcerations
juvenile dermatomyositis with lipodystrophy is a chronic systemic autoimmune disease characterised by early-onset proximal weakness and characteristic skin rashes, associated with loss of subcutaneous fat in a localised or generalised distribution, frequently with resultant metabolic abnormalities such as insulin resi...
juvenile dermatomyositis with myocarditis is an early-onset systemic inflammatory disorders affecting the skeletal muscles, the skin, and the heart. symptomatic cardiac involvement is uncommon in acute disease, but up to 50% of dermatomyositis patients will have asymptomatic cardiac involvement on non-invasive testing...
juvenile dermatomyositis with necrotising cutaneous vasculitis type of idiopathic inflammatory myopathy that is characterized by rash accompanied by muscle weakness, anorexia, and fatigue. juvenile form presents more insidiously with muscle weakness, particularly of the neck flexors, often preceded by fatigue and low ...
amyopathic juvenile dermatomyositis juvenile-onset amyopathic dermatomyositis is an uncommon variant of juvenile-onset dermatomyositis (jdm), characterised by the hallmark cutaneous features of dermatomyositis for at least 6 months without clinical or laboratory evidence of muscle disease. cutaneous calcinosis, vascul...
juvenile dermatomyositis with calcinosis is a rare childhood systemic autoimmune disease characterised by proximal muscle weakness, rashes due to chronic muscle and skin inflammation, and calcinosis due to the abnormal deposition of insoluble calcium salts within the skin, subcutaneous tissue, myofascia, or muscle. th...
juvenile dermatomyositis with overlap to nonorgan specific systemic autoimmune disorders juvenile dermatomyositis with overlap to non-organ specific systemic autoimmune disorders is an early-onset systemic inflammatory disorders affecting the skeletal muscles, the skin, and other organs, associated with other autoimmu...
polymyositis is an inflammatory muscle disease of unknown aetiology occurring predominantly in adults and characterised clinically by proximal muscle weakness (shoulders, arms, thighs), often with associated myalgia. involvement of pharyngeal and oesophageal muscles may result in dysphagia and a risk of aspiration pne...
anti-synthetase syndrome with polymyositis is an inflammatory muscle disease associated with antisynthetase autoantibodies, being the anti-jo-1 autoantibodies the most common anti-synthetase autoantibody. these patients have a constellation of symptoms, including myalgias, muscle weakness, and a combination of “core” ...
juvenile polymyositis is a rare childhood idiopathic inflammatory myopathy. it is frequently misdiagnosed, as it lacks a unique clinical phenotype. traditionally, it presents with weakness of the proximal muscles that evolves over weeks to months. the primary histologic features are fibre size variability, scattered n...
juvenile polymyositis, necrotising juvenile necrotizing polymyositis is an early-onset immune inflammatory muscle disorders characterised by high ck level, moderate to severe muscle weakness of acute or subacute onset and with histological features of muscle fibre necrosis mediated by macrophages as the main effector ...
juvenile polymyositis, paraneoplastic juvenile paraneoplastic polymyositis is an early-onset immune inflammatory muscle disorders associated with cancer. lung and colorectal cancers were the most common cancers in men; among women, 20% is related to breast neoplasia
juvenile polymyositis with anti-synthetase auto-antibody is a rare childhood idiopathic inflammatory myopathy associated with anti-synthetase autoantibody, being the anti-jo-1 the most common autoantibody. these patients have a constellation of symptoms, including myalgias, muscle weakness, and a combination of "core"...
juvenile polymyositis with interstitial lung disease is a rare childhood idiopathic inflammatory myopathy complicated by a serious pulmonary complication commonly seen in patients with polymyositis (pm) and dermatomyositis (dm). the presence of interstitial lung disease affects the prognosis and requires a more aggres...
juvenile polymyositis with overlap to non-organ specific systemic autoimmune disorder is a rare childhood idiopathic inflammatory myopathy associated with other autoimmune or connective tissue disorders, such as systemic sclerosis and mixed connective tissue disease.
juvenile polymyositis with pm-scl auto-antibody is a systemic autoimmune myopathy characterised by skeletal muscle weakness, typical rashes and other systemic features associated with anti-polymyositis-scleroderma pm-scl autoantibodies. these autoantibodies are commonly seen in juvenile myositis overlapping with anoth...
necrotising polymyositis is an immune inflammatory muscle disorders characterised by high ck level, moderate to severe muscle weakness of acute or subacute onset and with histological features of muscle fibre necrosis mediated by macrophages as the main effector cell. there are no t cell infiltrates or mhc-i expressio...
polymyositis with overlap to nonorgan specific systemic autoimmune disorders polymyositis with overlap to non-organ specific systemic autoimmune disorders is an idiopathic inflammatory myopathy associated with other autoimmune or connective tissue disorders, such as systemic sclerosis and mixed connective tissue disea...
paraneoplastic polymyositis paraneoplastic is a rare cancer associated entity. it presents sub-, or acutely with proximal weakness, often including the neck flexors, dysphagia, rarely the respiratory muscles and the heart are involved. sometimes muscle pain or myalgia occur. myopathology shows a targeted, cell-mediate...
non-hodgkin’s lymphoma, lung, and bladder carcinoma are the most frequently observed associated cancer types.
focal myositis is an idiopathic inflammatory myopathy that appears clinically like a muscular tumefaction that is solitary, painful, bleeds occasionally and is usually located in the musculature of the lower limbs. its rapid enlargement simulates that of a soft tissue tumour. this disorder may remit spontaneously, rem...
extraocular myositis is a focal myositis limited to extraocular muscles. patients usually present with orbital or periorbital pain, diplopia, abnormal extraocular motion, and proptosis. it can be seen alone or in association of other diseases.
inclusion body myopathy (ibm) is distinguished from polymyositis (pm) and dermatomyositis (dm) on the basis of clinical and histopathological features. a characteristic clinical phenotype is characterised by insidious onset of muscle weakness over months to years, muscle weakness localised predominantly in the thigh m...
inflammatory inclusion body myositis inclusion body myositis (ibm) is the most common idiopathic inflammatory myopathy after age 50. it typically presents with chronic insidious proximal leg and/or distal arm asymmetric muscle weakness leading to recurrent falls and loss of dexterity. creatine kinase is up to 15 times...
sporadic inclusion body myositis inclusion body myositis (ibm) is classified in the group of primary myositis (or inflammatory myopathies) characterised by inflammation of the striated muscles. it is a very progressive disease with muscular weakness that predominates in proximal muscles, although distal muscles can be...
hereditary inclusion body myositis hereditary inclusion-body myopathies (hibms) defines a group of rare muscle disorders with autosomal recessive or dominant inheritance and presence of muscle fibres with rimmed vacuoles and collection of cytoplasmic or nuclear 15-21 nm diameter tubulofilaments as revealed by muscle b...
noninflammatory inclusion body myopathy (ibm) is an idiopathic muscle disorder without inflammatory exudates and expression of class i major histocompatibility complex. rimmed vacuoles and “ibm-like” filaments without inflammatory cells are described in muscle biopsy.
hereditary inclusion body myopathy group of rare muscle disorders with autosomal recessive or dominant inheritance and presence of muscle fibres with rimmed vacuoles and collection of cytoplasmic or nuclear 15-21 nm diameter tubulo-filaments as revealed by muscle biopsy. the most common form of hereditary inclusion-bo...
desmin-related myopathy with mallory body-like inclusions familial or sporadic muscle disorders morphologically defined by intrasarcoplasmic aggregates of desmin, the intermediate filament of muscle cells, although several other proteins accumulate in these disorders. the mallory body-like form presented with neonatal...
hereditary inclusion body myopathy - joint contractures - ophthalmoplegia hereditary inclusion body myopathy type 3 is characterised by congenital joint contractures (normalizing during early childhood), external ophthalmoplegia, and proximal muscle weakness. in adult cases, the muscular weakness is progressive.
inclusion body myopathy with paget disease of bone and frontotemporal dementia is described as autosomal dominantly inherited multisystem degenerative disorder caused by mutations in p97/vcp (valosin-containing protein). myopathy is present in 90%of affected individuals, characterised by adult-onset, proximal and dista...
x-linked myopathy with excess autophagy x-linked myopathy with excessive autophagy is a childhood-onset x-linked myopathy characterised by slow progression of muscle weakness and unique histopathological findings: excessive autophagic activity and exocytosis of the phagocytosed material.
sporadic inclusion body myopathy sporadic inclusion body myositis is the most frequent acquired myopathy of middle and later life and is distinguished from other inflammatory myopathies by its selective pattern of muscle involvement and slowly progressive course, and by the combination of inflammatory and degenerative...
systemic sclerosis is a systemic disorder of the connective tissue; manifested by hardening and thickening of the skin, by abnormalities involving the microvasculature and larger vessels, and by fibrotic degenerative changes in various body organs including the heart, lungs, kidneys, and gastrointestinal tract. (arthr...
paediatric onset systemic sclerosis systemic sclerosis arising before the age of 16. involvement of internal organs is less common but arthritis and myositis are more common than in adults.
diffuse systemic sclerosis diffuse cutaneous systemic sclerosis (dcssc) is a subtype of systemic sclerosis (ssc) characterised by truncal and acral skin fibrosis with an early and significant incidence of diffuse involvement (interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myoc...
limited systemic sclerosis combination of calcinosis, raynaud phenomenon, oesophageal dysfunction, sclerodactyly and telangiectasia.
overlap or undifferentiated nonorgan specific systemic autoimmune disease nonorgan specific systemic autoimmune diseases which do not fulfil the diagnostic criteria for any single recognised disease entity.
antisynthetase syndrome (ass) is characterised by an inflammatory myositis associated with interstitial lung disease (ild) and antisynthetase antibodies. other symptoms, including arthritis, raynaud's phenomenon and mechanic's hands, are also associated with ass. several antisynthetase antibodies have been described, ...
igg4 related disease igg4 related syndrome (igg4-related disease: igg4-rd) is a clinical disease characterised by elevated serum igg4 concentration and tumefaction or tissue infiltration by igg4-positive plasma cells. the diagnostic criteria for igg4 related syndrome have been proposed, and it may be present in a cert...
primary cutaneous plasmacytosis is described as a skin disorder resulting from focal or multifocal dense infiltration of the skin by plasma cell aggregates. it may be associated with high levels of serum igg4. it typically presents as widespread reddish-brown papules, nodules and pigmented indurated plaques involving t...
benign lymphocytic infiltration of the skin benign lymphocytic infiltration of jessner is a chronic benign t-cell lymphoproliferative disorder characterised by the presence of non-scarring red tumid nodules, usually on facial skin. it may be difficult to distinguish from cutaneous lupus erythematosus.
lymphocytoma cutis is a benign, cutaneous b-cell lymphoproliferative disorder. it presents as papules, nodules or plaques usually on the head and neck and pursues a chronic course. it occurs as a response to known or unknown antigenic stimuli that result in the accumulation of lymphocytes and other inflammatory cells.
borrelial lymphocytoma cutis is a form of cutaneous lyme borreliosis seen principally in europe and most commonly linked to borrelia afzelii. it typically presents as a solitary nodule or plaque, usually located on the earlobe in children and on the areola of the breast or on the genitals in adults. it is characterise...
type 1 igg4 related autoimmune pancreatitis this is an inflammation of the pancreas that is the pancreatic manifestation of immunoglobulin g4-associated systemic diseases.