Castleman’s disease
The majority of this disease presents with painless lymph node enlargement. In patients with the focal form, pathology is more common in the hyaline vascular type. The enlarged lymph nodes can occur at any site, with fewer extra-nodal tissue lesions; they can be solitary or multiple aggregates, some of which may fuse. In patients with the polycentric type, the peak incidence is 50-60 years of age, and women are more common. The pathology is mainly plasmacytic or mixed. The common clinical manifestations are multiple, scattered lymph node enlargement, often accompanied by systemic symptoms such as fever, anemia, and wasting, and physical signs are mostly accompanied by hepatosplenomegaly. Laboratory tests may include anemia, accelerated sedimentation, increased globulin, polyclonal hypogammaglobulinemia, granulocytosis, and increased bone marrow plasma cell ratio. Other rare related clinical manifestations include: hematological manifestations such as refractory anemia, autoimmune hematocrit, lupus anticoagulant and thrombotic thrombocytopenic purpura; renal manifestations such as nephrotic syndrome, glomerulonephritis and acute renal failure; cutaneous manifestations such as common aspergillosis, skin pigmentation and characteristic multiple violet skin nodules; neurological manifestations such as pseudotumoral encephalopathy and peripheral neuropathy; amyloidosis, PO Neurological manifestations such as pseudotumor cerebri, peripheral neuropathy, amyloidosis, POEMS syndrome, etc.
Adult Still’s disease
The main manifestations are chills, high fever, mild enlargement of lymph nodes and liver and spleen, and transient red papules, while muscle and joint pains are not obvious. Leukocytosis, predominantly neutrophilic. Blood sedimentation is fast, but no obvious foci of infection can be found. The general condition is good. The blood was negative for rheumatoid factor, antinuclear antibodies and lupus cells. Antibiotic treatment was ineffective and adrenocorticotropic hormone treatment had a good effect. Most of the patients had a good prognosis, except for a few years when joint deformities could occur, but they could relapse. The final diagnosis needs to exclude lymphoma, chronic hepatitis and connective tissue diseases such as SLE as an exclusionary diagnosis.
Leukoarthrosis
(1) Oral ulcers Most of the lesions in the oral cavity appear as recurrent small ulcers, which are basically the same as recurrent mouth sores, and only a few are deep ulcers. The ulcers can occur on the lips, tongue, cheeks, palate, and gums, and generally heal in about 10 days.
(2) Ocular lesions may manifest as conjunctivitis, keratitis, chorioretinitis and retinitis, and more serious ones include iridocyclitis and pus accumulation in the anterior chamber. Optic neuritis and optic nerve atrophy can lead to vision loss and even blindness.
(3) Genital lesions In males, they are usually found in the scrotum and penis, and can also cause parametritis. In females, ulcers occur more often in the labia. The size of the ulcers is similar to or deeper than oral ulcers, and the pain is obvious. The interval between episodes is usually much longer than that of oral ulcers, ranging from a few months to a few years.
(4) Skin lesions Nodular erythema, folliculitis, boils, and ulcers appear on the skin, and may also manifest as erythema multiforme.
Among the four basic symptoms mentioned above, oral ulcer episodes are the most frequent, with most reports being over 90% and some reports even 100% having oral ulcers (mouth sores), and more than half of them are first-episode symptoms. Oral ulcers can appear simultaneously or alternately with other symptoms, and there are also cases where oral ulcers recur for several years or more than 10 years before other symptoms appear, and there are also cases where other symptoms appear earlier than oral ulcers. About 1/3 of the skin lesions are the first symptoms of the disease.
Dry syndrome
The onset of the disease is slow, most of them are weak, and there may be fever.
(1) Dry mouth, dry mouth and desire to drink, severe cases have difficulty in eating dry food. Due to low saliva and reduced flushing effect, tooth decay easily occurs, 63% of primary SS patients have tooth decay. 40% of patients have enlarged salivary glands, symmetrical, smooth surface, not hard, gland enlargement is sustainable or recurrent, rarely secondary infection. If the gland is hard and nodular, malignant change should be alerted.
(2) Dry eyes Patients have a sensation of rubbing foreign bodies in the eyes or no tears, and the lacrimal glands are usually not enlarged, or mildly enlarged.
(3) Skin mucosa Dry, scratchy skin, common rash is purpura, there are also urticaria-like rash in the form of polymorphic, nodular erythema. There are dry and cracked lips and mouth, oral ulcers, dry nasal and vaginal mucosa.
(4) Joints and muscles 70% to 80% have arthralgia and even arthritis, but destructive arthritis is rare. Muscle weakness may occur, and myositis may occur in 5%.
(5) Kidney Nearly half of the cases are complicated by renal damage, and the common site of involvement is the distal renal tubules, with symptomatic or subclinical renal tubular acidosis. The former shows low blood pH and inability to acidify urine (pH > 6). The subclinical type can be measured by ammonium chloride loading test.
Complications of renal tubular toxicity include.
(i) periodic hypokalemic paralysis.
(ii) renal chondromalacia.
(iii) nephrogenic uremia with proximal tubular involvement manifested by amino aciduria, phosphaturia, glycosuria, and β2-microglobulinuria. Glomerular involvement is rare, but the prognosis is very poor.
(6) Lung 17% of patients have dry cough without recurrent pulmonary infections. 50% of patients have excessive inflammatory cells in bronchopulmonary washings, indicating the presence of alveolar inflammation, and only a minority of patients develop diffuse interstitial pulmonary fibrosis. Pulmonary function tests often show decreased function, but most have no clinical symptoms.
(7) Digestive system
(i) atrophic gastritis with hyposecretion of gastric acid and lack of gastric acid.
(ii) Small intestine malabsorption.
(③) low exocrine function of the pancreas.
④ hepatomegaly, elevated serum transaminases, and liver pathology biopsy often shows changes of chronic active hepatitis in those with jaundice, and such patients respond better to epi-corticosteroids.
(8) Nervous system The symptoms include epilepsy, hemiplegia, hemianopia, myelitis, cerebral neuritis, and peripheral neuritis due to vasculitis at inaccessible sites. The latter involves mainly sensory fibers.
(9) Blood picture 1/4 to 1/5 show leukopenia and thrombocytopenia, and only a few have bleeding.
(10) Lymphatic tissue hyperplasia 5%-10% of patients have enlarged lymph nodes. At least 50% of patients have a large infiltration of lymphocytes in the internal organs during the course of the disease. More strikingly, the incidence of lymphoma is 44 times higher in patients with this disease than in the normal population. Two of the 150 cases of SS in our country presented with lymphoma during follow-up. SS may be preceded by macroglobulinemia or monoclonal hyperglobulinemia. After the development of lymphoma, blood immunoglobulin levels may decrease and autoantibodies disappear.