Rheumatic diseases are a group of diseases that affect bones, joints and surrounding soft tissues, such as tendons, bursae, and fascia, in general, and have various causes, such as infectious, immune, metabolic, endocrine, degenerative, geographic environmental, and genetic.
Rheumatic diseases are characterized by pain (joint, muscle, soft tissue, nerve, etc.) as the main symptom, and arthritis due to various causes is an important component, but rheumatic diseases are limited to arthritis. The term “connective tissue disease” or “collagen disease” used in the past is part of rheumatic diseases, and they are not exactly the same as “rheumatic diseases”.
The pathological changes in rheumatic diseases are diverse and involve interstitial tissues throughout the body, and connective tissue is the most important site of lesions in rheumatic diseases, and both dense connective tissue, such as cartilage and tendons, and lax connective tissue can have extensive damage of varying degrees. Sparse connective tissue damage is characterized by mucinous edema, fibrinoid degeneration, granuloma formation, inflammatory cell infiltration, and late hyaline or sclerotic changes.
Immune injury plays an important role in the pathogenesis of rheumatic diseases, and many rheumatic diseases are, at least in part, due to tissue damage caused by immune abnormalities. Immune injury can be divided into four basic types: allergic type I reactions, which can be local or systemic; antibody-mediated type II reactions, which are characterized by the combination of antibodies with otherwise cell surface antigens or antigens absorbed on the cell surface; immune complex type III reactions, which are characterized by the local deposition of immune complexes on the cell or tissue surface; and cell-mediated type IV reactions, which are the result of direct contact between sensitized T cells and specific antigens. These types are not mutually exclusive and can co-exist in some patients.
Advances in immunogenetics and studies of HLA antigens and related diseases have increased the understanding of the pathogenesis of rheumatic diseases. Many of the rheumatic diseases associated with MHC genes are autoimmune, and the gene encoded by HLA-B27 is strongly associated with ankylosing spondylitis.
Classification of rheumatic diseases
A more comprehensive classification of rheumatic diseases is as follows;
I. Diffuse connective tissue diseases
(a) Rheumatoid arthritis;
(ii) Juvenile rheumatoid arthritis systemic onset (Still’s disease), polyarticular onset, oligoarticular onset.
(C) Systemic lupus erythematosus.
(IV) Systemic sclerosis (progressive systemic sclerosis, scleroderma)
(V) polymyositis with dermatomyositis
(vi) necrotizing vascular and other vasculitis, polyarteritis nodosa (including arteritis complicated by viral hepatitis B and allergic granulomatosis, i.e., Churg-Strauss vasculitis), allergic vasculitis (including Henoch-Schonlein purpura), hypocomplementemic vasculitis, Wegner’s granulomatosis, giant cell arteritis (temporal arteritis. Takayasn arteritis), mucocutaneous lymph node syndrome (Kawasaki disease), leukoaraiosis, cryoglobulinemia, juvenile dermatomyositis.
(vii) dry syndrome
(viii) Overlap syndrome (including undifferentiated and mixed connective tissue diseases).
(ix) Others include rheumatic polymyalgia, lipofuscinosis (Weber Christiun disease), erythema nodosum, recurrent chondromalacia, eosinophilic diffuse fasciitis, adult-onset Still’s disease.
Second, the arthritis complicating spondylitis
(A) Ankylosing spondylitis.
(B) Reiter’s syndrome.
(C) psoriatic arthritis.
(D) Inflammatory bowel disease arthritis.
Third, degenerative joint disease (osteoarthritis, osteoarthrosis)
(A) primary (including erosive osteoarthritis).
(B) secondary.
Fourth, infection-induced arthritis, tenosynovitis and bursitis
(i) Directly caused (1) Bacterial, including
① Gram-staining positive cocci such as staphylococci.
② Gram-staining negative cocci such as gonococci, etc.
③ Gram-staining negative bacilli, ③ Gram-staining negative bacilli
④Acid-resistant bacilli.
⑤ Spirochetes including Lyme disease.
(6) Other bacteria such as leprosy;
(2) Mycoplasma sexually.
(3) Viral including viral hepatitis.
(4) fungal, and
(5) Parasitic.
(6) Unknown cause or suspected infectious such as Whipple’s disease.
(II) Indirectly caused (reactive)
(1) Bacterial, including
(1) acute rheumatic fever
(ii) short-circuiting of the intestine.
(iii) post-dysentery-Shigella.
(4) Yersinia pestis and other bacteria;
(2) viral (hepatitis B)
V. Metabolic diseases and endocrinopathies with rheumatic disease manifestations
(a) Crystal
(1) sodium urate (gout)
② pyrophosphate dihydrate (pseudogout, chondrocalcinosis)
(iii) apatite and other alkaline calcium phosphates.
④ oxalate.
(II) Biochemical abnormalities
①amyloidosis.
②Vitamin C deficiency (scurvy).
(iii) Specific enzyme deficiencies (including Fabry disease, Farber disease, etc.)
④ hyperlipidemia (type I, II, IV, etc.)
⑤ Mucopolysaccharidoses.
⑥Hemoglobin abnormalities (SS disease, etc.), ⑦True connective tissue disease
(vii) true connective tissue diseases (Ehler-Danlas disease, Marfan disease, osteogenesis imperfecta, elastic pseudoxanthoma, etc.)
⑧ hemochromatosis.
(⑨) hepatomegaly (Wilson’s disease), (⑩)
(⑩褐黄病, (11) Gaucher’s disease and others.
(C) endocrinopathies
①Diabetes mellitus.
(ii) acromegaly.
(3) hyperparathyroidism
(iv) thyroid disease (hyperthyroidism, hypofunction, thyroiditis), (v) other.
⑤ others.
(iv) Immunodeficiency diseases
① primary immunodeficiency.
② acquired immunodeficiency syndrome (AIDS).
(E) other genetic diseases
① congenital polyarticular curvature.
(ii) hyperactivity syndrome.
(iii) progressive ossifying myositis.
VI. Tumors
(a) Primary (such as synovial tumor, synovial sarcoma, etc.).
(ii) metastatic.
(C) multiple myeloma.
(iv) Leukemia and lymphoma.
(V) Choroidal nodular synovitis.
(F) Osteochondroma.
(vii) Others.
VII. Neurological diseases
(A) Neurological arthropathy.
(II) Compressive nerve disorders
(i) carpal tunnel syndrome.
(ii) Nerve root disease.
(iii) Spinal stenosis.
(c) Sympathetic reflex atrophy.
(iv) Other.
Eight, there are joint manifestations of bone, periosteal and cartilage diseases
(A) Bone thinning
(i) circumferential.
(ii) limited (site-specific, transient).
(ii) Osteochondrosis.
(iii) Hypertrophic osteoarthropathy.
(iv) Diffuse primary bone hypertrophy (including ankylosing vertebral hypertrophy-Forestier disease).
(v) Osteoarthritis
(i) circumscribed (deformational osteitis-Paget’s disease).
② limited (iliac dense osteitis, osteitis pubis).
(vi) Osteonecrosis.
(vii) Osteochondritis (detached osteochondritis).
(viii) Bone and joint dysplasia.
(ix) Slipped epiphysis.
(X) Costochondritis (including Tietze syndrome).
(xi) Osteolysis and chondrolysis.
(XII) osteomyelitis.
IX. Non-articular rheumatic diseases
(I) myofascial pain syndrome
(I) circumscribed (fibromyalgia, fibromyalgia).
② confined.
(B) low back pain and intervertebral disc disease.
(iii) tendonitis (tenosynovitis) and/or bursitis
(i) subacromial and subdeltoid bursitis, ①
②biceps tendonitis.
③Tendonitis.
④Hawksbill bursitis.
⑤Internal and external epicondylitis
⑥De Quervain’s tenosynovitis
⑦Adherent shoulder bursitis.
⑧Trigger finger
⑨ Others.
(iv) Tenosynovial cysts.
(v) Fasciitis.
(F) Chronic ligament and muscle strain.
(vii) vasodilator disorders
(i) Erythromelalgia.
(ii) Raynaud’s disease or phenomenon.
(viii) Other pain syndromes (including climate allergy, psychogenic rheumatism).
(i) Diseases often complicated by arthritis
(i) trauma (as a result of direct trauma), (ii)
(ii) internal disorders of the joints.
(iii) pancreatic disease.
(iv) sarcoma-like tumors.
⑤ Recurrent rheumatism.
(6) intermittent joint effusion
⑦Erythema nodosum.
⑧ Hemophilia.
(B) Other diseases
(1) multicentric reticuloendotheliosis (nodular lipofuscinosis).
(ii) Familial Mediterranean fever.
③Goodpasture syndrome.
④Chronic active hepatitis.
(⑤) pharmacogenic rheumatic syndrome.
(6) Dialysis-associated syndrome.
(7) Foreign body synovitis.
⑧Purulent acne and sweat gland inflammation.
(9) palmar and metatarsal muscle pustulosis.
⑩Sweet syndrome, (11) others.
Clinical symptoms
Most rheumatic diseases have a chronic course, and the clinical manifestations of the same disease may vary considerably among individuals or at different times. The course of the disease is recurrent and remitting.
I. Pain syndrome Joint, muscle and tendon pain is quite common and can involve large and small joints of the extremities, with symmetrical joint pain being the most common. Morning stiffness and Raynaud’s sign are important concomitant symptoms. The onset, nature, location, duration, presence or absence of systemic symptoms and age of onset of pain vary from patient to patient. For example, the onset of gout is sudden and acute, and pain in the toe and foot joints is common. Rheumatoid arthritis is slow to develop and affects the wrist, metacarpophalangeal and proximal interphalangeal joints and the cervical spine. Ankylosing spondylitis, on the other hand, almost invariably begins with low back pain and progresses upward, affecting the peripheral joints, mostly the large joints of the lower extremities as well. Systemic lupus erythematosus has more pronounced peripheral systemic manifestations in addition to arthralgia. Some patients eventually develop joint stiffness, deformity, and loss of function, while some patients have recurrent joint swelling and pain but no deformity in the end.
Skin manifestations Most patients have skin changes, which are specific or non-specific. There are various manifestations, such as urticaria, erythema annulare, erythema papulosum, erythema multiforme, erythema nodosum, erythema facialis, etc. The pathological basis of the skin lesions is vasculitis, the most important of which is leukocytoclastic vasculitis. The size of the involved vessels, the intensity of the reaction, the duration, the extent of involvement and the pathological changes vary according to the different skin lesions.
Ocular symptoms may precede systemic symptoms by several months or years. Some become prominent in the course of the disease, the lesions may involve the cornea, retina, pigment layer, symptoms include ocular dryness, increased intraocular pressure, cataract, orbital myositis, ocular muscle paralysis, vision loss or even blindness.
Pulmonary manifestations Respiratory distress is a common complaint, and the causes include pneumonia, eosinophilic pulmonary infiltration, pulmonary hemorrhage, focal granuloma formation, fibrosing alveolitis, interstitial pneumonia and pleural effusion.
V. Gastrointestinal system manifestations Because the basic pathological changes are extensive small-vessel vasculitis, the involvement of the digestive system is also extensive, such as gastrointestinal bleeding, perforation or intestinal obstruction, which can be life-threatening.
Cardiovascular system manifestations Myocardium, endocardium, pericardium, conduction system, arteries and veins can be involved. Clinical manifestations include enlarged heart, accelerated heart rate, systolic murmur in the heart valve area, pericardial friction sound, high blood pressure and various arrhythmias, and in severe cases, heart failure.
Renal manifestations Renal lesions are quite common, including interstitial inflammation, fibrosis, membranous nephropathy, glomerular basement membrane thickening, amyloidosis, etc. Floaters, polyuria or oliguria, proteinuria, hypertension and acute and chronic renal failure are present.
VIII. Others There may be hemolytic anemia, thrombocytopenia, oral ulcers, parotid enlargement, otitis media, pigmentation disorders, etc. Laboratory tests
I. Blood picture There is often mild to moderate anemia, and the anemia is aggravated when combined with hemolysis. Some patients have leukocytopenia and/or thrombocytopenia, or complete blood count.
Anti-nuclear antibody (ANA) refers to a class of antibodies against various nuclear components, which are divided into different kinds of ANAs due to different nuclear antigens, such as anti-nuclear protein antibody (anti-DNP antibody); anti-DNA antibody; including two major categories, namely anti-single-stranded DNA (denatured DNA) antibody (anti-ss-DNA antibody) and anti-double-stranded DNA (anti-DNA antibody). ) and anti-double-stranded DNA (natural DNA) antibodies (anti-ds-DNA antibodies); anti extractable nuclear antigen antibodies (anti-ENA antibodies) include anti nuclear glycoprotein antibodies (anti-RNP antibodies) and anti-Sm antibodies; anti-RNA antibodies; anti cytoplasmic antibodies and anti nuclear antigen antibodies.
Rheumatoid factor RF is an autoantibody to macroglobulin that reacts with the antigenic determinants of the Fc fragments of denatured or agglutinated lgG molecules. RF is mainly of lgM type, but also of lgG and lgA types. In rheumatic diseases, rheumatoid arthritis RF detection rate is the highest, about 70-90%, systemic lupus erythematosus, sclerosis, mixed connective tissue disease, dry syndrome can also be positive. Other positive findings such as viral infections, parasitic infections, chronic inflammation, tumor radiotherapy and chemotherapy, etc..
Other blood sedimentation is often increased, C-reactive protein is positive, hypocomplementemia, hyperuricemia, immune complex is positive. Immunoglobulins in the blood are increased or decreased. Depending on the degree of organ damage involved, there are changes in urine, renal function, cardiac function, etc. In rheumatic diseases there are characteristic or non-characteristic changes in joint X-rays, which must be considered in combination with clinical data and laboratory tests.
Treatment
The aim of treatment for rheumatic diseases is to improve the symptoms and change the disease, and to stop the progress. The most widely used drugs to improve symptoms are non-steroidal drugs, such as anti-inflammatory pain, ibuprofen, naproxen and fenpropathrin. Adrenocorticotropic hormone should be applied early, especially in the combination of heart, brain, lung, kidney and other important organ lesions, can quickly relieve the disease, hormone in remission should be gradually reduced, reduce the dose too quickly will cause re-aggravation of the disease.
The drugs to improve the condition are penicillamine, gold preparation, ralston and immunosuppressants, which are selected appropriately according to different diseases, different individuals and different conditions, i.e. emphasizing the individualization of treatment.