The concept of<unk> <unk>  came into existence in 1980 when LeRoy et al found out some disease entities which don't qualify the criteria of definite connective tissue disease but resemble rheumatological diseases. The patients with<unk>  presents with signs and symptoms of connective tissue diseases like arthritis, Raynaud's phenomenon, Dry-mouth, dry eyes.Undifferentiated connective tissue disorder<unk>  are actually a group of disorders that are unclassified in other connective tissue disorders.<unk>  may be classified according to criteria developed by American College of Rheumatology(ACR) and European League Against Rheumatism(EULAR) : early Raynaud's phenomenon, early inflammatory arthritis that is not classified as rheumatoid arthritis, some manifestations same as that of inflammatory myopathy, systemic lupus erythematosus, Sjögren's syndrome, vasculitis, serositis, or interstitial lung disease which don't meet diagnostic criteria for each of them and early scleroderma.The exact pathogenesis of<unk>  connective tissue disorder<unk>  is not fully understood. It is understood that<unk>  is the result of autoimmune process and occurs in phases: Initial phase: which is asymptomatic and absence of auto-antibodies and second phase with the presence of auto-antibodies. The second phase is usually triggered by the environmental factors, such as infection. Auto-antibodies appear before the symptoms of the disease.The time between the two is variable. Autoantibodies usually seen in<unk>  with positive correlation are antibodies against C1q ,Anti heat shock protein(hsp)-65, Anti hsp 60. The clinical picture involves the overlap of symptoms of other rheumatological disease but not completing the criteria of their diagnosis. More studies are needed to find the pathogenesis of<unk> .The cause of<unk>  has not been identified.UCTD is differentiated with other causes of arthritis and rash such as SLE, Sjögren's syndrome, Rheumatoid arthritis. Not enough epidemiological studies have been done but about 25% of the presentation of more than 1 year resembles connective tissue disorder.UCTD is differentiated with other causes of arthritis and rash such as SLE, Sjögren's syndrome, Rheumatoid arthritis.There are no established risk factors for<unk> .The symptoms of<unk> <unk>  usually are similar to connective tissue diseases but couldn't complete the criteria for their definitive diagnosis. If left untreated, 24.3-33% of patients with<unk>  may progress to develop manifestations of well defined connective tissue disease such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome. Common complications of<unk>  include Interstitial lung disease- Non specific interstitial pneumonia, atherosclerosis. Depending on the extent of the<unk>  at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. The presence of interstitial lung disease is associated with a particularly good prognosis among patients with<unk>  rather than being idiopathic.Patients with<unk>  <unk>  may have a positive history of Raynaud's phenomenon, arthritis, photosensitivity, sicca symptoms, pleuritis/pericarditis. The common symptoms of<unk>  include arthralgia, dry eyes, rash, photosensitivity and dry mouth. Less common symptoms of<unk>  include fever, pleuritic chest pain and seizures. Patients with<unk>  usually appear well but can present with low-grade fever, Raynaud's phenomenon, rash and Erythema nodosum in the skin examination. On examining HEENT,Conjunctivitis, Uveitis, Keratoconjunctivitis sicca, dry-mouth can be seen. In the neck, lymphadenopathy can be seen. Rhonchi, Wheezing, pleural friction rub and Pericardial friction rub can be heard on auscultation.On examining abdomen, tenderness in the right/left upper/lower abdominal quadrant and hepatomegaly are noted. In the back, tenderness over lumbar vertebrae can occur and sacral edema may be seen.Rashes can be seen in the genitourinary exam. The patient can have altered mental statuswith psychosis and peripheral neuropathy and Pitting/non-pitting edema of the lower extremities. Laboratory findings suggestive of<unk>  include Leukopenia,increased erythrocyte sedimentation rate, C-reactive protein, Antinuclear antibodies, anti DNA antibodies, anti Ro/SSA antibodies and anti-RNP antibodies.The pharmacologic medical therapy is recommended based on the rheumatic disease pattern manifested by the patient such as methotrexate in those exhibiting arthritis and dermatitis. Supportive therapy includes; avoiding cold exposure in those experiencing Raynaud's phenomenon, avoiding sun in photosensitivity, non-steroidal anti-inflammatory drugs for pain control, sunscreen used for photosensitivity, and emollients for dry skin, rash.The mainstay of treatment for<unk>  connective tissue disorder is medical therapy. Surgery is rarely needed for patients for surgical biopsy in case of diagnosis.The feasibility of surgery depends on the prognosis at diagnosis.
Undifferentiated connective tissue disease
Mixed connective tissue disease
Connective tissue disease
Acrokeratoelastoidosis of Costa
Primary cutaneous amyloidosis
Fibromuscular dysplasia
Scleroderma
MSBD syndrome